Handbook of Pediatric Orthopedics Second Edition
Handbook of Pediatric Orthopedics Second Edition
Paul D. Sponseller, MD Professor and Head Children's Orthopaedics Johns Hopkins Medical Institutions Baltimore, Maryland
Thieme New York • Stuttgart
Thieme Medical Publishers, Inc. 333 Seventh Ave. New York, NY 10001 Executive Editor: Kay D. Conerly Managing Editor: J. Owen Zurhellen IV Editorial Assistant: Tess Timoshin Editorial Director: Michael Wachinger Production Editor: Print Matters, Inc. International Production Director: Andreas Schabert Vice President, International Marketing and Sales: Cornelia Schulze Chief Financial Officer: James W. Mitos President: Brian D. Scanlan Compositor: The Manila Typesetting Co. Printer: Sheridan Books, Inc Medical Illustrator: Hong Cui, MD Front cover illustration: William and Henry Sponseller Back cover illustration: Nina Sponseller Library of Congress Cataloging-in-Publication Data Sponseller, Paul D. Handbook of pediatric orthopedics / Paul D. Sponseller. – 2nd ed. p. ; cm. Includes bibliographical references and index. ISBN 978-1-58890-517-8 (alk. paper) 1. Pediatric orthopedics–Handbooks, manuals, etc. I. Title. [DNLM: 1. Orthopedics–Handbooks. 2. Child. 3. Infant. WS 39 S763h 2010] RD732.3.C48S66 2010 618.92’7–dc22 2010025229 Copyright © 2011 by Thieme Medical Publishers, Inc. This book, including all parts thereof, is legally protected by copyright. Any use, exploitation, or commercialization outside the narrow limits set by copyright legislation without the publisher’s consent is illegal and liable to prosecution. This applies in particular to photostat reproduction, copying, mimeographing or duplication of any kind, translating, preparation of microfilms, and electronic data processing and storage. Important note: Medical knowledge is ever-changing. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy may be required. The authors and editors of the material herein have consulted sources believed to be reliable in their efforts to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibility of human error by the authors, editors, or publisher of the work herein or changes in medical knowledge, neither the authors, editors, nor publisher, nor any other party who has been involved in the preparation of this work, warrants that the information contained herein is in every respect accurate or complete, and they are not responsible for any errors or omissions or for the results obtained from use of such information. Readers are encouraged to confirm the information contained herein with other sources. For example, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this publication is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is of particular importance in connection with new or infrequently used drugs. Some of the product names, patents, and registered designs referred to in this book are in fact registered trademarks or proprietary names even though specific reference to this fact is not always made in the text. Therefore, the appearance of a name without designation as proprietary is not to be construed as a representation by the publisher that it is in the public domain. Printed in the United States 54321 ISBN 978-1-58890-517-8
To all whom I have been privileged to teach in the combined mission of caring for growing children: You have been a source of continuous instruction.
Contents
Foreword..................................................................................................ix Peter O. Newton Preface.......................................................................................................xi Contributors......................................................................................... xiii 1 Anatomy and Normal Development in Children..................... 1 Paul D. Sponseller 2 Disorders of Skeletal Growth and Development...................55 Paul D. Sponseller 3 Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics............................................................134 Paul D. Sponseller 4 Neuromuscular Disorders in Pediatric Orthopedics.................................................................................. 164 Paul D. Sponseller and Thomas O. Crawford 5 Pediatric Trauma.........................................................................174 Paul D. Sponseller 6 Normal Values and Medications..............................................210 Paul D. Sponseller and Philip R. Neubauer 7 Common Procedures..................................................................224 Paul D. Sponseller Index.......................................................................................................247
Foreword
This revised second edition of the Handbook of Pediatric Orthopedics is densely packed with vital information about pediatric orthopedics yet is presented in a manner that makes it a useful study guide as well as a valuable reference. Dr. Sponseller has put together a handbook–this word in the title is fitting–that every student and resident rotating on a pediatric orthopedic surgery service should have in their lab coat pocket at all times. In addition it should come out at night to prepare for the following day’s clinic and surgical cases. Read it and use it, and you will have a solid foundation in pediatric orthopedics. This book uniquely houses the essentials of pediatric orthopedics and is primarily targetted at the novice. The text begins with the basic development of the musculoskeletal system and progresses to the common orthopedic pathologies that effect children. The book is relevantly referenced and is filled with the facts, graphs, charts, and tables that are so frequently a part of everyday pediatric orthopedic practice. For example, the evaluation of lower extremity growth is accompanied by the contribution of growth from each of the lower limb growth plates as well as the methods and graphs to calculate growth remaining. These are critical values in planning for corrective procedures, and the several accepted methods for doing so are clearly presented and easy to comprehend. This is but one example of the method of presenting the critical data in an easily usable format. For the more common conditions, the details are present that will allow a reasonable understanding, while for the more obscure pathologies, the prose is appropriately brief or absent. Common things being common, this book contains what everyone who cares for (or plans to care for) children needs to know. There are several groups of readers who will find the information contained in the Handbook of Pediatric Orthopedics particularly helpful. Those who are making their very first entry into the world of pediatric orthopedics often find our specialty initially overwhelming. It is either seen as orthopedics with growth factored into the equation and a myriad of syndromes, or else as pediatrics with too many bones to remember and x-rays that are daunting to interpret. Whether coming from either of these two perspectives, this handbook provides a source of information that is easily digestible and that is certain to quickly provide calm and order. It is just right for the rotating medical student and equally appropriate for residents starting
their initial pediatric orthopedic experience. It also remains a resource for those beyond the introductory phase to the specialty, potentially for years to come due to the key sources of classification systems, scoring schemes, and graphical data particularly related to skeletal growth. As such, fellows in pediatric orthopedics may also want to refer back to this text at various times in the year (and beyond). Be ready for rounds, for clinic, for your attending’s questions, and most importantly for what you need to know in order to care for your patients on the pediatric orthopedic service. Read this handbook and keep it close by. There is little doubt it will serve you well. Congratulations and thank you to Dr. Sponseller for bringing us the second edition of this valuable, straightforward, and concise Handbook of Pediatric Orthopedics. Peter O. Newton, MD Clinical Associate Professor Department of Orthopaedic Surgery University of California–San Diego Division of Orthopaedic Surgery Rady Children’s Hospital San Diego, California
Preface
The practice of pediatric orthopedics relies heavily on knowledge of principles and on technical skills. Nevertheless, there is also a need for the use of data and standards. In the process of teaching and working with professionals of all levels of training in children’s orthopedics, I have tried to compile information that is essential for the practice of our specialty. I gathered these essentials into a practical handbook that is complete enough to be useful. The book is not meant to provide definitive coverage of individual problems or theoretical principles. Rather, it is intended to provide factual information to which we must commonly refer. The first chapter, Anatomy and Normal Development in Children, gives norms for osseous development, motor development, innervation, and growth patterns and predictions. Chapter 2, Disorders of Skeletal Growth and Development, follows and provides radiographic parameters and clinical standards for treatment. Chapter 3, Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics, is included because many of these conditions are rare and not immediately familiar to the orthopedist; an overview is given with references at the end. Chapter 4, Neuromuscular Disorders in Pediatric Orthopedics, covers the basics of many of these unusual conditions. After an important chapter on Pediatric Trauma (Chapter 5), Chapter 6, Normal Values and Medications used in pediatric orthopedics, presupposes a knowledge of their pharmacology but gives dosages based on weight as well as their route and frequency. Normal laboratory values for children are listed by age. Last, Chapter 7, Common Procedures, including blocks, traction, special casts, aspiration, and arthrograms, is meant to provide technical information that is not readily available elsewhere. It is my hope that this handbook will be a practical aid in the day-to-day practice of pediatric orthopedics for those at all levels of experience. I will be pleased to receive any comments or suggestions for the eventual third edition at [emailprotected].
u Acknowledgments I thank those I have been privileged to help train for making me constantly examine what might otherwise be taken for granted. I also thank my family—Amy, Matt, and Nina—for their inspiration. The careful editing of Sara Cleary, Elaine Henze, Kay D. Conerly, and J. Owen Zurhellen IV and the clear, concise drawings of Hong Cui, MD, have made this book possible.
Contributors
Thomas O. Crawford, MD
Associate Professor Division of Pediatric Neurology Johns Hopkins Medical Institutions Baltimore, Maryland
Philip R. Neubauer, MD, PharmD Assistant Professor Department of Orthopaedic Surgery Johns Hopkins Medical Institutions Baltimore, Maryland
Paul D. Sponseller, MD
Professor and Head Children's Orthopaedics Johns Hopkins Medical Institutions Baltimore, Maryland
1 Anatomy and Normal Development in Children 1
1 Anatomy and Normal Development in Children Knowledge of normal growth and development is important for evaluation and diagnosis. This chapter contains relevant anatomy and developmental norms. It also contains a description of normal gait and guidelines for interpreting a gait study.
u Neurodevelopmental Norms When evaluating a patient at risk of developmental delay, it is helpful to have norms to determine whether a delay is present. The section below presents the chronological appearance of certain key motor, social, and language skills. The Denver II Developmental Screening Test displays these in graphic form. Although the orthopedic surgeon will not perform the test formally, it provides an excellent summary of developmental milestones. Table 1.1 provides the norms for milestones. Information on the Denver II Developmental Screening Test is available from the following:
Denver Developmental Materials Inc. PO Box 371075 Denver, CO 80237 E-mail: [emailprotected]
The Denver II Developmental Screening Test can be used with children from birth to age 6 years. It is used to decide which children should be
Table 1.1 Norms for Motor Milestones Skill
Mean Age (mo)
St. Dev.
Roll from back to stomach
3.6
1.4
Roll from stomach to back
4.8
1.4
Sit tailor-style
5.3
1.0
Sit unsupported
6.3
1.2
Crawl (many never crawl)
7.8
1.7
Pull to stand
8.1
1.6
Cruise
8.8
Walk
11.7
2
Run
15
3
1.7
Source: Palmer F, Capute A. Keys to developmental assessment. In McMillan J, ed. Oski’s Pediatrics, 3rd ed. Philadelphia: Lippincott Williams & Wilkins; 2006:789
2 Handbook of Pediatric Orthopedics
referred for diagnostic evaluation. It is not an IQ test. It has four sections: personal–social, fine motor, language, and gross motor. The Denver II scoring chart provides an excellent summary of motor development.
Psychomotor Skills in Children During Years 1 Through 5 l l l l l l l l
l l l l l
Neonatal period (first month) Supine: Generally flexed and tone a little low 2 Months Prone: Head sustained in plane of body in ventral suspension Social: Smiles on social contact 4 Months Supine: Reaches and grasps objects and brings them to mouth Sitting: No head lag on pull to sitting position 4 to 6 Months Prone: Rolls over to supine Semantics: Turns to his or her own name 7 Months Sitting: Sits briefly with support of pelvis Adaptive: Transfers objects from hand to hand 10 Months Standing: Pulls to standing position Motor: Creeps or crawls 12 to 18 Months Syntax: Speech generally consists of single-word utterances (comment) 12 Months Motor: Walks with one hand held, “cruises” or walks holding on to furniture Language: Two “words” besides mama and dada 15 Months Motor: Walks alone, crawls up stairs 18 Months Motor: Runs stiffly Social: Feeds self 24 Months Motor: Opens doors Syntax: Uses two- and three-word combinations (telegraphic speech) 30 Months Motor: Jumps 36 Months Motor: Goes up stairs alternating feet, stands momentarily on one foot
1 Anatomy and Normal Development in Children 3 l l
48 Months Motor: Hops on one foot, throws ball overhand 60 Months Motor: Skips
Bibliography Richter SB, Howard BJ, Sturner R. Normal infant and childhood development. In McMillan J, ed. Oski’s Pediatrics. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2006:593–601
Referral Criteria Referral should be made if the infant is displaying any of the following: 1. Not rolling by 6 months 2. Not sitting independently by 8 months 3. Handedness develops too early (by 12 months): May indicate abnormality of opposite side 4. Not walking by 18 months 5. No words by 14 months
u Neurologic Anatomy Sensation Knowledge of dermatomes will help in the evaluation of neurologic conditions. There is, however, some variation and overlap between levels. Injury to a single nerve root may not produce complete loss of sensation within a dermatome. Sensation should be recorded as increased, decreased, absent, or dysesthetic. The sensation of proprioception and vibration is carried in the dorsal column of the spinal cord: light touch in the ventral spinothalamic tract and pain and temperature in the lateral spinothalamic tract. During neurologic root recovery, pain sensation returns before light touch (Fig. 1.1).
Upper Extremity Motor Examination Injury to the roots or the cord of the cervical spine follows certain patterns. Even though most muscles have innervation from multiple segments, each root has specific muscles and sensory regions for which it is critical. The diagram below is helpful for diagnosing cervical root lesions and spinal cord injury. Motor testing can be performed in one coordinated sequence, from proximal to distal: deltoid (C5), biceps (C5), wrist extension (C6),
4 Handbook of Pediatric Orthopedics
Fig. 1.1 Dermatomes.
finger extension (C7), finger flexion (C8), finger abduction and adduction (T1) (Fig. 1.2).
Upper Extremity Muscle Innervation Because most muscles are innervated by multiple segments, it is necessary to know all the roots controlling a given muscle. Fig. 1.3 indicates the roots contributing to a given muscle in the upper extremity. For purposes of strength grading, the following five-grade scale has been widely used:
l
Grade 1: Flicker Grade 2: Less than antigravity Grade 3: Maintains position against gravity Grade 4: Moves against submaximal resistance
l
Grade 5: Full strength
l l l
1 Anatomy and Normal Development in Children 5
Fig. 1.2 Sensory and motor innervation C6 to T1. Note: Only sensory loss is shown in the shaded hand, and only muscle involvement is shown in the arm. (From McQueen JD, Khan MI. Neurologic evaluation. In Sherk HH, Dunn EJ, Eismont FJ, et al, eds. The Cervical Spine. 2nd ed. Philadelphia: J.B. Lippincott; 1989: 206 (Fig. 4-5). Reprinted with permission.)
6 Handbook of Pediatric Orthopedics
Fig. 1.3 Innervation of muscles of upper extremity.
Formation of the Brachial Plexus Unless one works with the brachial plexus constantly, it is difficult to remember its anatomy accurately. The anatomy is depicted here to understand injuries from birth and later trauma. Most traction injuries involve the upper roots (Fig. 1.4).
1 Anatomy and Normal Development in Children 7
Fig. 1.4 Formation of the brachial plexus.
Peripheral Nerve Testing in the Upper Extremity In evaluating an injury of the upper extremity in children (such as for supracondylar fracture), it is important to have methods to test the peripheral nerves: the median nerve may be tested by grip or finger flexion and the anterior interosseous nerve (a branch of the median that can be selectively injured) by testing distal interphalangeal flexion of the index finger and thumb, forming an “O.” l
l
Radial nerve: By extending the thumb, the wrist or the metacarpophalangeal joints. Ulnar nerve: By crossing fingers, abducting fingers, or flexing the distal interphalangeal joint of the fifth finger (Fig. 1.5).
Lower Extremity Motor Innervation Knowledge of lower extremity motor innervation is important for understanding spina bifida, lumbar disk herniation, spinal cord injury, and other conditions. Innervation of muscles is by descending spinal segments at
8 Handbook of Pediatric Orthopedics
Fig. 1.5 Documentation of the status of all nerves and circulation before treatment of supracondylar humerus fractures. This involves (A) checking active palmarflexion (median nerve); (B) flexion of distal interphalangeal joints of the index finger and thumb–anterior interosseous nerve; (C) dorsiflexion of the metacarpophalangeal joints–posterior interosseous nerve; (D) flexion of the fifth finger distal interphalangeal joint; or (E) crossing of index and second fingers– ulnar nerve. progressively distal levels of the limb, with the notable exception of gluteus maximus, medius, and minimus (L5 through S2). The most important motors to know are iliopsoas (L1 through L3), adductors (L2 through L4), quadriceps (L2 through L4), hamstrings (L4 through L5), anterior tibialis (L4 through L5), gastrocnemius (S1), and glutei (L5 through S2) (Fig. 1.6).
1 Anatomy and Normal Development in Children 9
Lumbar 1
Sacral 2
3
4
5
1
2
3
PSOAS PECTINEUS ADDUCTORS QUADRICEPS OBT. EXT. TIBIALIS ANT TIBIALIS POST TENSOR FASCIA LATA GLUTEUS MED & MIN SEMIMEMBRANOSUS + TENDINOSUS EXT HALL LONGUS EXT. DIG. PERONEUS TERTIUS PERONEUS BREVIS PERONEUS LONGUS GASTROCNEMIUS SOLEUS BICEPS FEMORIS GLUTEUS MAXIMUS FLEX. HALLUCIS FLEX. DIGITORUM FOOT INTRINSICS
Fig. 1.6 Segmental innervation of muscles of the lower limb.
u Skeletal Development Appearance of Secondary Ossification Centers and Physeal Closure In many situations it is important to know whether an epiphysis should be ossified at a given age, such as in evaluating a patient with a hip dislocation, skeletal dysplasia, or elbow fracture. Normal times for appearance and closure of the long bones are given in Fig. 1.7; for the hand and foot, see Fig. 1.8. The following are some important milestones: 1. The distal femoral epiphysis is the first to ossify, at ~39 weeks’ gestation; the proximal tibia ossifies 1 week later.
10 Handbook of Pediatric Orthopedics
Fig. 1.7 Age of appearance of secondary ossification centers (A) and physeal closure (B) in the long bones. (From Ogden JA. Radiologic aspects. In Ogden JA, ed. Skeletal Injury in the Child. 2nd ed. Philadelphia: W.B. Saunders; 1990:84 (Figs. 3-28 and 3-29). Reprinted with permission.)
1 Anatomy and Normal Development in Children 11
Fig. 1.7 (continued)
12 Handbook of Pediatric Orthopedics
Fig. 1.8 Age of appearance of secondary ossification centers and physeal closure in the hand (A) and foot (B). ([A] from O’Brien ET. Fractures of the hand and wrist region. In Rockwood CA Jr, Wilkins KE, King RE, eds. Fractures in Children. 3rd ed. Philadelphia: J.B. Lippincott Co., 1991: 320 (Fig. 4-1). Reprinted with permission. [B] from Aitken JT, Causey G, Joseph J, Young JZ. The foot. In Aitken JT, Causey G, Joseph J, Young JZ, eds. A Manual of Human Anatomy. Vol IV, Lower Limb. 2nd ed. Edinburgh: E & S Livingstone; 1966:80 (Fig. 30). Reprinted with permission.) 2. The mean time for ossification of the proximal femoral epiphysis is 4 months, but normal may be up to 11 months. The greater trochanter ossifies at 4 to 6 years. 3. The triradiate cartilage closes before Risser I. 4. The tarsal navicular does not ossify until 3 to 4 years, so its location must be inferred from the position of the first metatarsal. 5. The last physis to close is that of the medial clavicle, at age 20 to 25 years.
1 Anatomy and Normal Development in Children 13
Fig. 1.8 (continued) 6. The sequence of ossification about the elbow can be remembered by the mnemonic CRITOE (Fig. 1.9): l l l l l l
C apitellum (age 2) R adial head (age 5) I nternal epicondyle (age 7) T rochlea (age 9) O lecranon (age 10) E xternal epicondyle (age 11)
14 Handbook of Pediatric Orthopedics
Fig. 1.9 Appearance of age of ossification centers about the elbow can be summarized by mnemonic CRITOE. (From Sponseller PD. Orthopaedic injuries. In Nichols DG, Yaster M, Lappe DG, Buck JR, eds. Golden Hour: The Handbook of Advanced Pediatric Life Support. St. Louis: Mosby Year Book; 1991:350 (Fig. 18-3). Reprinted with permission.)
7. Angle of distal humeral articular surface: This angle is key to understanding any angular change about the elbow. It is best measured by the Baumann angle, between the humeral shaft and the lateral condylar physis (Fig. 1.10). Its normal value is 72 ± 4 degrees. There is no difference between sexes or ages from 2 to 13 years. 8. Determination of skeletal age using the elbow: This can be determined by the olecranon or modified Sauvegrain method (Fig. 1.11). The five stages include (1) bipartite olecranon, (2) half-moon–shaped olecranon, (3) rectangular olecranon, (4) partially fused, or (5) fully fused. They occur at 6-month intervals of skeletal age from 11 years in girls and 13 in boys. The fully fused olecranon occurs at skeletal age of 13 in girls and 15 in boys and marks the deceleration of growth velocity. 9. Determination of skeletal age using the Modified Tanner–Whitehouse III Skeletal Maturity Assessment (Table 1.2 and Fig. 1.12) Patients with curves over 30 degrees at stage 3 to 4 are at high risk of progression to surgery, and patients with curves less than 20 degrees at this stage are at low risk.
1 Anatomy and Normal Development in Children 15
Fig. 1.10 Differing configuration of the distal humerus and landmarks used for measurement of the Baumann angle.
5 fused 4 partially fused 2 half-moon
3 rectangular
bipartite Fig. 1.11 Simplified Sauvegrain method for skeletal maturity assessment.
Table 1.2 Key Findings of the Simplified Tanner–Whitehouse III Skeletal Maturity Assessment Key Features
Tanner-Whitehouse III Stage
Greulich and Pyle Reference
Related Maturity Signs
1. Juvenile slow
Digital epiphyses not covered
Some digits are at stage E or lower
Female 8 year + 10 months, male 12 year + 6 months (note fifth middle phalanx)
Tanner stage 1
2. Preadolescent slow
All digital epiphyses covered
All digits at stage F
Female 10 year, male 13 year
Tanner stage 2, starting growth spurt
3. Adolescent rapid–early
Preponderance of digits are capped. The 2nd through 5th metacarpal epiphyses wider than their metaphyses
All digits are at stage G
Female 11 and 12 year, male 13 year + 6 months and 14 year
Peak height velocity, Risser stage 0, open pelvic triradiate cartilage
4. Adolescent rapid–late
Any distal phalangeal physes are clearly beginning to close
Any distal phalanges are at stage H
Female 13 year (digits 2, 3, 4), male 15 year (digits 4,5)
Girls typically in Tanner stage 3, Risser stage 0, open triradiate cartilage
5. Adolescent steady–early
All distal phalangeal physes closed; others are open
All distal phalanges and thumb metacarpal are at stage I; others remain at stage G
Female 13 year + 6 months, male 15 year + 6 months
Risser stage 0, triradiate cartilage closed, menarche only; occasionally starts earlier
6. Adolescent steady–late
Middle or proximal phalangeal physes are closing
Middle or proximal phalanges are at stages H and I
Female 14 year, male 16 year (late)
Risser sign positive (stage 1 or more)
7. Early mature
Only distal radial physis is open; metacarpal physeal scars may be present
All digits are at stage I. The distal radial physis is at stage G or H
Female 15 year, male 17 year
Risser stage 4
8. Mature
Distal radial physis completely closed.
All digits are at stage I
Female 17 year, male 19 year
Risser stage 5
Source: From Sanders JO, Khoury JG, Kishan S, et al. Predicting scoliosis progression from skeletal maturity: a simplified classification during adolescence. J Bone Joint Surg Am. 2008;90(3):541 (Table 1). Reprinted with permission.
16 Handbook of Pediatric Orthopedics
Stage
1 Anatomy and Normal Development in Children 17
A
C
B
D
Fig. 1.12 Simplified Tanner–Whitehouse III method for skeletal maturity assessment. Note that the distal phalangeal epiphyses are the key to the curve acceleration phase (CAP). When they are capped, the phase is beginning, and when they are all closed, the phase has ended. (A) Stage 1: All digital epiphyses not covered (epiphyses not as wide as metaphyses). (B) Stage 2: All digital epiphyses are covered. (C) Stage 3: Most epiphyses cap their metaphyses. Capping is a small bend over the metaphyseal edge. This is also the beginning of the CAP. (D) Stage 4: At least one of distal phalanges closed. (From Sanders JO, Khoury JG, Kishan S, et al. Predicting scoliosis progression from skeletal maturity: a simplified classification during adolescence. J Bone Joint Surg Am. 2008;90(3):540-553 (Figs. 1-B, 2-B, 3-B, 4-B). Reprinted with permission.)
18 Handbook of Pediatric Orthopedics
Cervical Spine Radiographic Normal Values for Children Alignment 1. The cervical spine in children is characterized by increased mobility at C2–3, termed pseudosubluxation, which should not exceed 3 mm. 2. The tip of the odontoid should not be more than 1 cm from the basion of the skull (anterior rim of the foramen magnum). 3. The physis of the odontoid normally fuses between 3 and 6 years. 4. The atlas–dens interval should be less than 4 mm. 5. The Power ratio is the ratio of the distance from basion to posterior arch of C1 divided by the distance from the opisthion to the anterior arch of C1. This ratio should be less than 1. 6. The retropharyngeal space should not exceed 8 mm; if greater, it could signify bleeding from a fracture or a dislocation.
Fig. 1.13 Normal values of cervical spine alignment for children. (From Sponseller PD. Orthopaedic injuries. In Nichols DG, Yaster M, Lappe DG, Buck JR, eds. Golden Hour: The Handbook of Advanced Pediatric Life Support. St. Louis: Mosby Year Book; 1991:353 (Fig. 18-4). Reprinted with permission.)
1 Anatomy and Normal Development in Children 19
7. The spinal laminae should form a smooth line posteriorly. 8. The vertebral bodies may be wedged anteriorly, especially on their superior surfaces, until age 10 (Fig. 1.13).
Development of the Cervical Spine First Cervical Vertebra (Atlas) 1. Body: Not ossified at birth; the center (occasionally two centers) appears during the first year after birth; the body may fail to develop, and forward extension of neural arches may take its place. 2. Neural arches: Appear bilaterally at approximately the seventh fetal week; most of the anterior portion of superior articulating surface is usually formed by the body. 3. Synchondrosis of spinous processes: Unites by the third year. Union rarely is preceded by the appearance of a secondary center within the synchondrosis. 4. Neurocentral synchondrosis: Fuses at approximately the seventh year (Fig. 1.14).
Second Cervical Vertebra (Axis) 1. Body: One center (occasionally two) appears by the fifth fetal month. 2. Neural arches: Appear bilaterally by fetal month 7. 3. Neural arches fuse posteriorly by the year 2 or 3.
Fig. 1.14 Axial, coronal, and sagittal views of the developing atlas. The first cervical vertebra is formed by three ossification sites: the anterior arch (gray), or centrum, and the two neural arches (white). (From Oh BC, Wang MY. Cervical anatomy and surgical approaches. In Kim DH, Betz RR, Huhn SL, Newton PO, eds. Surgery of the Pediatric Spine. New York: Thieme; 2008:95 (Fig. 8-1). Reprinted with permission.)
20 Handbook of Pediatric Orthopedics
4. Bifid tip of spinous process: Occasionally a secondary center is present in each tip. 5. Neurocentral synchondrosis: Fuses at 3 to 6 years 6. Inferior epiphyseal ring: Appears at puberty and fuses at around 25 years 7. “Summit” ossification center for odontoid: Appears at 3 to 6 years and fuses with the odontoid by 12 years 8. Odontoid (dens). Two separate centers appear by fetal month 5 and fuse with each other by month 7 9. Synchondrosis between odontoid and neural arch: Fuses at 3 to 6 years 10. Synchondrosis between odontoid and body: Fuses at 3 to 6 years 11. Posterior surface of body and odontoid (Fig. 1.15)
Spinal Growth The growth of the spine occurs relatively earlier than that of the extremities, but not to the extreme of cranial growth. If arthrodesis is considered in a growing child, the following rules will help predict the consequences for growth.
Guidelines and Rules of Thumb 1. T1–S1 growth rates l l l
0 to 5 years: 2 cm/year 6 to 10 years: 0.9 cm/year 10 years: 1.8 cm/year through growth spurt
Fig. 1.15 Coronal and sagittal views of the developing axis. There are four ossification centers present at birth: one center for each neural arch (white), one for the odontoid process (gray), and one for the body (black). (From Oh BC, Wang MY. Cervical anatomy and surgical approaches. In Kim DH, Betz RR, Huhn SL, Newton PO. Surgery of the Pediatric Spine. New York: Thieme; 2008:96 (Fig. 8-2). Reprinted with permission.)
1 Anatomy and Normal Development in Children 21
2. Growth of the T1–S1 segment is two thirds complete by 6 years. 3. Growth remaining from T1–S1 at age 5 is 15 cm. 4. DiMeglio has summarized the growth of the extremities and spine with respect to maturity indicators (elbow growth centers, Risser sign, menarche, triradiate cartilage closure) in Fig. 1.16. Triradiate cartilage closure slightly precedes the peak height velocity; olecranon closure occurs at the peak height velocity, and menarche occurs just after peak height velocity; all are at or before Risser sign turns to 1. Note that posterior arthrodesis alone does not stop growth completely within a fused segment. Further growth can cause relative compression of disk space, exacerbation of preexisting lordosis, or rotation of scoliosis (“crankshaft”) (Fig. 1.17).
Spinal Growth Remaining Calculation l
0.07 cm × number of spinal segments × growth years left (Fig. 1.18)
Fig. 1.16 Simplified skeletal age assessment with the olecranon method during the accelerating pubertal growth phase of peak height velocity and Risser grade 0 from the ages of 11 to 13 years in boys, with a decelerating growth phase after elbow fusion. Y cartilage closure = triradiate cartilage closure. (From Charles YP, Dimeglio A, Canavese F, Daures JP. Skeletal age assessment from the olecranon for idiopathic scoliosis at Risser grade 0. J Bone Joint Surg Am. 2007:89(12):2739 (Fig. 1). Reprinted with permission.)
22 Handbook of Pediatric Orthopedics Cm/Year
T1-L5 2
T1-T12
1
L1 L5
1
5
10
15 Years
Fig. 1.17 Approximate growth velocity of the spine by segments: thoracic, lumbar, and combined segments. Note that the greatest velocity is in preschool years, and then a significant drop occurs. (From Dimeglio A. Growth of the spine before age 5 years. J Pediatr Orthop B. 1992;1(2):103 (Fig. 7). Reprinted with permission.)
Extremity Growth: Relative Contributions to Growth of the Long Bones The contributions of each physis to longitudinal growth are a reflection of its activity, which in turn influences remodeling potential. A simple rule of thumb is that most growth occurs “away from the elbow and at the knee” in the upper and lower extremities, respectively (Fig. 1.19).
1 Anatomy and Normal Development in Children 23
Fig. 1.18 Growth remaining in the spine by segment for (A) boys and (continued on page 24)
24 Handbook of Pediatric Orthopedics
Fig. 1.18 (continued from page 23) (B) girls. Note that the remaining component of sitting height is due to the head, cervical spine, and cranium. (From Dimeglio A. Growth of the spine before age 5 years. J Pediatr Orthop B. 1992;1(2):104 (Figs. 11 and 12). Reprinted with permission.)
1 Anatomy and Normal Development in Children 25
Fig. 1.19 Relative contributions to growth of the long bones of the upper (A) and lower (B) extremity. (From Ogden JA. Radiologic aspects. In Ogden JA, ed. Skeletal Injury in the Child. 2nd ed. Philadelphia: W.B. Saunders; 1990:85 (Fig. 3-30A,B). Reprinted with permission.) Some guidelines for estimating growth: 1. At each physis, this estimation can be done during the preadolescent years using the approximate rates: l l l l
Proximal femur: ⅛ inch/year Distal femur: ⅜ inch/year Proximal tibia: ¼ inch/year 3 Distal tibia: /16 inch/year
These estimates are true until age 13 for girls or age 15 for boys. For tallerthan-average children, these rates are greater. For long growth periods of more than 3 years, it is better to consult growth tables (Fig. 1.20).
26 Handbook of Pediatric Orthopedics
Fig. 1.20 Growth remaining in normal distal femur and proximal tibia following consecutive skeletal ages. (From Anderson M, Green WT, Messner MB. Growth and predictions of growth in the lower extremities. J Bone Joint Surg Am. 1963;45(1):10 (Chart III). Reprinted with permission.)
1 Anatomy and Normal Development in Children 27
Fig. 1.21 Length of normal femur and tibia for boys (including epiphyses). (From Anderson M, Messner MB, Green WT. Distribution of lengths of the normal femur and tibia in children from one to eighteen years of age. J Bone Joint Surg Am. 1964;46(6):1201 (Chart II). Reprinted with permission.)
28 Handbook of Pediatric Orthopedics
Fig. 1.22 Length of normal femur and tibia for girls (including epiphyses). (From Anderson M, Messner MB, Green WT. Distribution of lengths of the normal femur and tibia in children from one to eighteen years of age. J Bone Joint Surg Am. 1964;46(6):1200 (Chart I). Reprinted with permission.)
1 Anatomy and Normal Development in Children 29
2. 3. 4. 5.
Total adult height = 2 × height at age 2. Total adult length of lower extremities = 2 × length at age 4. Growth ceases in girls at 15 to 15.5 years, in boys at 17 to 17.5 years. Another way to estimate adult height: Males = (Father’s height + mother’s height + 6 cm)/2. Females = (Father’s height + mother’s height – 6 cm)/2. Two standard deviations = ±5 cm.
l l l
6. Another way to estimate adult height is to use the multiplier method (Table 1.3, page 36). This takes into account the fact that existing height is a strong predictor of final height.
Growth Curves for Long Bones Growth curves are useful when absolute lengths are needed. Figs. 1.21 and 1.22 represent the same population data as presented differently in the “growth remaining” curves.
mm 50 mm 50 40 40 30 30 20 20 10 10
9
10
11
12
13
14
15 skeletal age (years)
A Distal tibia—boys. Calculated remaining growth (—— ±1 SD, — - — ±2 SD).
8
9
10
11
12
13
14
15 skeletal age (years)
B Distal tibia—girls. Calculated remaining growth (—— ±1 SD, — - — ±2 SD).
Fig. 1.23 Growth remaining in distal tibia (mean ± 2 SD) for boys (A) and girls (B). (From Karrholm J, Hansson LI, Selvik G. Longitudinal growth rate of the distal tibia and fibula in children. Clin Orthop Relat Res. 1984;191:124 (Figs. 2B and 3B). Reprinted with permission.)
30 Handbook of Pediatric Orthopedics
Growth Remaining Curves These are a reformulation of the data shown in Figs. 1.21 and 1.22. This format is especially useful for estimating the effects of physeal closure. One must know the patient’s skeletal age and percentile rank for height.
Longitudinal Growth from Distal Tibial and Fibular Physes 1. Distal tibial and fibular physeal fractures often occur in childhood and adolescence. Growth plate damage may occur. Physeal closure in either bone then must be evaluated for significance of both shortening and angular deformity. 2. The following growth-remaining graphs may be used for prediction of the deformity. The percentile to follow for a given patient may be obtained from the percentile of the patient’s rank in Figs. 1.21, 1.22, and 1.23. 3. Length lost from total arrest may be calculated. In general, inequality of less than 1 cm is of no clinical significance. 4. Prediction of angular growth disturbance as a result of peripheral arrest may be calculated from the growth remaining and the width of the physis. Because the average distal tibia is 4 to 5 cm wide, 10 degrees of angulation is likely to occur in boys before the age of 13½ and in girls before 11½. 5. This may also be used to predict the effects of surgical hemiepiphyseodesis.
Straight-Line Graph for Predictions of Discrepancy For growth disturbances that do not change characteristics over time, if skeletal age is known, the eventual inequality of limb length at maturity can be calculated by plotting data points on the straight-line graph developed by Moseley (Figs. 1.24 and 1.25). The procedure is explained step by step. Conditions for which the graph may not be appropriate are for those with a phasic nature, for example, a discrepancy that is due to juvenile rheumatoid arthritis or Klippel-Trenaunay syndrome.
1 Anatomy and Normal Development in Children 31
90
1 80
2
Leg Length - cms.
m
at
ur
it y
mean
3 4 5 6 7 8 9 10 1112 1314
Skeletal Age - Girls
70
50
40
Leg Length - cms.
60
REFERENCE ia SLOPES tib al m i ur ox pr fem l a t dis h bot E LL
80 70 60 50
G
A
RM
NO
30
20
1
2
3
4
Skeletal Age - Boys 5 6
7 8 9 10 11 12 13 14 1516
m
at
ur
it y
mean
Fig. 1.24 Moseley straight-line graph for predicting limb-length inequality. (From Moseley CF. A straight-line graph for leg-length discrepancies. J Bone Joint Surg Am. 1977;59(2):176 (Fig. 1). Reprinted with permission.)
32 Handbook of Pediatric Orthopedics
At each visit to the hospital obtain these three values:
1. The length of the normal leg measured by or thoroentgenogram from the most superior part of the femoral head to the
middle of the articular surface of the tibia at the ankle. 2. The length of the short leg, and 3. The radiologic estimate of skeletal age.
Drwa a vertical line through that point the
Place the point for the normal leg on the ' normal leg' line at the appropr – iate length.
entire height of the graph and throught the skeletal age 'scalar' area of either boys or girls as the case
Place the point for the short leg on the current skeletal age line at the correct length.
maybe. This line repre – sents the current skeletal
age.
From that point draw a line parallel to the reference slope for the particular growth plates fused. This is the new grwoth line for the normal leg.
Ascertain the length of the normal leg just prior to surgery, and mark that point on the normal leg line. Reference slopes
The growth plates each make a known contribution to the total growth of the leg.
Distal femur – 37%
65% – both
Proximal Tibia – 28% Plot successive sets of three points in the same
Mark the point where the current skeletal age line interesects that sloping 'scalar' in the skeletal age area which corresponds to the radiologic estimate
fashion.
The percent age decrease in slope of the new growth line (taking the previous slope as 100%) exactly represents the loss of the contribution of the fused growth plate(s).
Draw the straight line which best fits the points
plotted previously for successive lengths of the short leg.
of skeletal age.
is represented by the vertical distance between the two growth lines. is represented by the difference in slope between the two growth lines, taking the slope of the normal leg as 100%
Draw the horizontal straight line which best fits the points plotted previously in the skeletal
Extend to the right the growth line of the short leg.
age area.
is represented by the position of that horizontal line and indicates whether the
child is 'taller' or 'shorter' that the mean. is represented by the intersections of this horizontal line with the scalars in the
Draw the new growth line for the lengthened leg exactly parallel to the previous growth line but displaced upwards by distance exactly
equal to the length increase achived. Since the growth plates are not affected neither is the growth rate, and the slope of the line is therefore unchanged.
Project the growth line of the short leg to intersect the maturity line, taking into account the effect of a lengthening procedure if necessary.
From the intersection with the maturity line draw a line whose slope is equal to the reference slope for the proposed surery.
The point at which this line meets the growth line of the normal leg indicates the point at which the surgery should be done. Note that this point is defined, not in terms of the calendar, but in terms of the length of the normal leg. Since lenthening procedures do not affect the rate of growth, the timing of this procedure is not critical and will be governed by clinical considerations.
skeletal age area.
The Maturity Point is the intersection of the line with the maturity scalar.
Maturity Through the maturity point draw
a vertical line, the Maturity line. This line represents
point. Anticipated discrepancy at maturity.
maturity and the cessation of
growth. Its intersections with
the growth lines of the two legs
represents their anticipated lengths at maturity.
In keeping a child’s graph up to date it is recommended that these lines be draw in pencil. The addition of futher data makes this method more accurate and may require slight changes in the positions of these lines.
Draw the new growth line of the normal leg as shown in section'C'.
Data is plotted exactly as before except that the length of the short leg is plotted first and is placed on the growth line previously established for the short leg.
Fig. 1.25 Instructions for the Moseley graph. (From Moseley CF. A straight-line graph for leg-length discrepancies. J Bone Joint Surg Am. 1977;59(2):177 (Fig. 2). Reprinted with permission.)
1 Anatomy and Normal Development in Children 33
Upper Extremity Growth Growth arrest occurs less commonly in the upper extremity. Occasionally, however, as a result of infection, tumor, or trauma, growth is affected, and it may become necessary to calculate the resulting discrepancy (Fig. 1.26).
Fig. 1.26 Growth remaining from major upper extremity physes. These graphs are from the Child Research Counsel, Denver, Colorado. Mean (solid line) and two standard deviations (dotted lines). (From Bortel DT, Pritchett JW. Straightline graphs for the prediction of growth of the upper extremities. J Bone Joint Surg Am. 1993;75(6):889 (Fig. 2). Reprinted with permission.)
34 Handbook of Pediatric Orthopedics
Overall Physical Growth Norms Physical growth norms include mean stature for age and their respective percentiles. They are useful in screening for growth disturbances and estimating height at maturity (Figs. 1.27 and 1.28).
Fig. 1.27 Age 2 to 20 years: Boys stature-for-age and weight-for-age percentiles.
1 Anatomy and Normal Development in Children 35
Fig. 1.28 Age 2 to 20 years: Girls stature-for-age and weight-for-age percentiles.
Table 1.3 Multiplier Method for Height (Paley Method)* Age (yr)
Height Multiplier
Table 1.4 Multiplier Method for Lower Limb (LL) Lengths† Age (yr + mo)
LL Length Multiplier
Girls
Boys
Birth
3.30
3.53
Birth
5.080
4.630
0.5
2.50
2.64
0+3
4.550
4.155
1.0
2.22
2.34
0+6
4.050
3.725
1.5
2.04
2.16
0+9
3.600
3.300
2.0
1.92
2.04
1+0
3.240
2.970
2.5
1.81
1.94
1+3
2.975
2.750
3.0
1.73
1.86
1+6
2.825
2.600
3.5
1.68
1.78
1+9
2.700
2.490
4.0
1.62
1.73
2+0
2.590
2.390
4.5
1.57
1.67
2+3
2.480
2.295
5.0
1.51
1.63
2+6
2.385
2.200
5.5
1.47
1.58
2+9
2.300
2.125
6.0
1.42
1.53
3+0
2.230
2.050
6.5
1.38
1.50
3+6
2.110
1.925
7.0
1.34
1.45
4+0
2.000
1.830
7.5
1.31
1.42
4+6
1.890
1.740
8.0
1.28
1.38
5+0
1.820
1.660
8.5
1.25
1.35
5+6
1.740
1.580
9.0
1.23
1.32
6+0
1.670
1.510
9.5
1.21
1.30
6+6
1.620
1.460
10.0
1.18
1.28
7+0
1.570
1.430
10.5
1.16
1.26
7+6
1.520
1.370
11.0
1.13
1.23
8+0
1.470
1.330
11.5
1.11
1.21
8+6
1.420
1.290
12.0
1.08
1.19
9+0
1.380
1.260
12.5
1.06
1.16
9+6
1.340
1.220
13.0
1.04
1.13
10 + 0
1.310
1.190
13.5
1.03
1.11
10 + 6
1.280
1.160
14.0
1.02
1.08
11 + 0
1.240
1.130
14.5
1.01
1.06
11 + 6
1.220
1.100
15.0
1.01
1.04
12 + 0
1.180
1.070
15.5
1.01
1.03
12 + 6
1.160
1.050
16.0
1.00
1.02
13 + 0
1.130
1.030
16.5
1.00
1.01
13 + 6
1.100
1.010
17.0
1.00
1.01
14 + 0
1.080
1.000
17.5
“
1.01
14 + 6
1.060
NA
18.0
“
1.00
15 + 0
1.040
NA
15 + 6
1.020
NA
16 + 0
1.010
NA
16 + 6
1.010
NA
17 + 0
1.000
NA
*The patient’s existing height may be multiplied by a number on this table corresponding to age to determine final height.
Boys
Girls*
Source: From Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg Am. 2000;82(10):1440 (Table 5). Reprinted with permission. Abbreviations: NA, not applicable. †The patient’s limb-length inequality may be multiplied by a number from this table corresponding to age to determine the final discrepancy. It may also be used to calculate the amount of growth remaining in the lower limb.
1 Anatomy and Normal Development in Children 37
The Multiplier Method for Growth Prediction The multiplier method can be used to predict the growth of long bones or the entire stature as long as the growth is occurring naturally without any intervention. It uses the patient’s own initial growth data and multiplies that by a figure known to be the proportion of growth remaining. Therefore, the growth of one limb or one bone can be calculated from just one measurement. If a growing patient has a limb-length discrepancy, that discrepancy is multiplied by the multiplier to calculate the final discrepancy. Tables 1.3 and 1.4 present the multipliers for different ages.
Alignment in the Transverse Plane 1. Femoral anteversion is the angle between the plane of the femoral head and neck and that of the posterior surface of the femoral condyles (Fig. 1.29). Femoral anteversion declines steadily in normal children from a mean of 25 degrees at birth to 15 degrees at adulthood (Fig. 1.30). It may be followed clinically by recording internal and external rotation of the hip in extension. It can be estimated in the clinic with the patient prone by internally rotating the hip until the greater trochanter is considered to be directly lateral and measuring the angle formed by the flexed tibia with the vertical (Fig. 1.31). Anteversion is most accurately measured by
Fig. 1.29 Femoral anteversion: definition.
38 Handbook of Pediatric Orthopedics
Fig. 1.30 Normal values for femoral anteversion. (From Shands AR Jr, Steele MK. Torsion of the femur. J Bone Joint Surg Am. 1958;40(4):806 (Graph I). Reprinted with permission.)
Fig. 1.31 Clinical measurement of femoral anteversion. This may be estimated with the patient prone by rotating the hip internally until the greater trochanter has reached maximal prominence and then noting the angle formed by the tibia with a vertical line. (Clinical acccuracy described in Ruwe PA, Gage JR, Ozonoff MB, DeLuca PA. Clinical measurement of femoral anteversion. J Bone Joint Surg Am. 1992;74:820–830 [original drawing]).
1 Anatomy and Normal Development in Children 39
Fig. 1.32 Measurement of anteversion by computed tomography. (A) Center of femoral head. (B) Center of base of femoral neck. (C) Condylar axis. (D) Anteversion is the angle between the two lines so defined. If the femoral head is posterior to the condylar axis, the value of the angle is negative and is termed retroversion. (From Murphy SB, Simon SR, Kijewski PK, Wilkinson RH, Griscom NT. Femoral anteversion. J Bone Joint Surg A.m 1987;69(8):1175 (Fig. 10). Reprinted with permission.)
computed tomography (CT). The method of Murphy is shown here (Fig. 1.32). Anteversion can also be measured by standard radiographs using techniques of Ogate or Magilligan. 2. CT measurement of anteversion: On sequential cuts by CT, a line is drawn through the centers of the femoral head and base of the neck. Another line is drawn through the posterior surfaces of the femoral condyles. The angle between these two lines is the femoral anteversion (Fig. 1.32).
Bibliography Magilligan DJ. Calculation of the angle of anteversion by means of horizontal lateral roentgenography. J Bone Joint Surg Am. 1956;38A(6):1231–1246 Murphy SB, Simon SR, Kijewski PK, Wilkinson RH, Griscom NT. Femoral anteversion. J Bone Joint Surg Am. 1987;69(8):1169–1176
3. Ogate method of determining femoral anteversion using biplane radiographs a. This method can be used when CT is not readily available. It uses graphs to provide trigonometric calculations of anteversion as well as true neck-shaft angle from two standardized plain radiographs (Figs. 1.33 and 1.34). It is built on the fact that the tibia is perpendicular to the condylar axis when the knee is flexed. The method is accurate when positioning is done carefully, to within ± 6 degrees for anteversion and ± 5 degrees for true neck-shaft angle.
40 Handbook of Pediatric Orthopedics
Fig. 1.33 Positioning for the “projected AP femur X-ray” in the method of Ogate. The projected neck-shaft angle obtained from this film is labeled α and used in the next step in Figs. 1.35 and 1.36 (see).
1 Anatomy and Normal Development in Children 41
Fig. 1.34 Positioning for the “projected lateral femur X-ray” in the method of Ogate. The tibia should be freely resting flat on the cassette. The projected neckshaft angle obtained from this film is labeled β and is used in the next step in Figs. 1.35 and 1.36 (see).
b. Technique for obtaining radiographs: The patient is positioned with the knee flexed 90 degrees and perpendicular to the surface of the radiography table. This places the condylar plane of the femur in a true horizontal position. A projected anteroposterior neck-shaft radiograph is taken, and the angle is drawn and labeled α. The patient is then positioned on the side, with the knee flexed and the tibia horizontal. A projected lateral neck-shaft radiograph is taken, and the angle is drawn and labeled β. c. Using the graphs in Figs. 1.35 and 1.36, values for true neck-shaft angle and anteversion can be obtained.
42 Handbook of Pediatric Orthopedics
Fig. 1.35 Determination of true anteversion of the femur using the intersection of projected anteroposterior (α) and lateral (β). (From Ogata K, Goldsand EM. A simple biplanar method of measuring femoral anteversion and neck-shaft angle. J Bone Joint Surg Am. 1979;61(6):849 (Fig. 5A). Reprinted with permission.)
Fig. 1.36 Determination of true neck-shaft angle. (From Ogata K, Goldsand EM. A simple biplanar method of measuring femoral anteversion and neck-shaft angle. J Bone Joint Surg Am. 1979;61(6):849 (Fig. 5B). Reprinted with permission.)
1 Anatomy and Normal Development in Children 43
Development of the Tibiofemoral Angle During Growth The coronal tibiofemoral angle changes dramatically during the first 5 years of life—from varus to excessive valgus to “normal” valgus. This was illustrated by Salenius in the graph redrawn in Fig. 1.37. It was made from clinical measurements on 1000 normal children. Radiographs are not generally necessary in the first 12 to 18 months of life. Chapter 2 details the interpretation of abnormal conditions.
Alignment of the Lower Extremity 1. Knowledge of normal tibiofemoral alignment is essential for planning osteotomies around the knee. Normal values vary slightly, depending on the width of the pelvis and limb lengths. 2. Definitions l
l
l l l
Mechanical axis is the angle of two lines between the centers of the hip, knee, and ankle; normal angle is 0 degrees, inclined 3 degrees from the vertical. Anatomic axis is the angle between the tibial and femoral diaphyses; normal is 6 degrees. The knee joint line should be horizontal. Femoral joint angle is 90 - (β + Θ) Tibial joint angle is 90 - Θ (Fig. 1.38)
Fig. 1.37 The tibiofemoral angle during growth. (From Salenius P, Vankka E. The development of the tibiofemoral angle in children. J Bone Joint Surg Am. 1975;57(2):260 (Fig. 1). Reprinted with permission.)
44 Handbook of Pediatric Orthopedics
Fig. 1.38 Alignment of lower extremity. In normal subjects, β = 6 degrees and Θ = 3 degrees; but these may vary depending on the distance between the hip centers, femoral neck-shaft angle, and limb length.
Fig. 1.39 Appearance of metatarsus adductus.
1 Anatomy and Normal Development in Children 45
Fig. 1.40 Quantification of metatarsus adductus. This is done with child prone. A line is drawn through the middle of the heel and examiner notes which toe it intersects. (From Bleck EE. Developmental orthopaedics. III: Toddlers. Dev Med Child Neurol. 1982;24(5):545 (Fig. 20). Reprinted with permission.)
Fig. 1.41 Measurement of the foot progression angle.
46 Handbook of Pediatric Orthopedics
Clinical Evaluation of Rotation of the Lower Extremities Rotational abnormalities can be approached in a systematic fashion. Foot progression angle quantifies the sum of rotations occurring in the femur, tibia, and foot. These components may be assessed by the parameters pictured on the following pages. Normal values throughout growth for all of these parameters are given, as determined by Staheli et al. Treatment for these rotational deformities is rarely indicated, but showing the natural progression to the parents in graphic form can be helpful. Metatarsal adduction and abduction (Figs. 1.39 and 1.40): Forefoot adduction is generally recorded from clinical measurements, but radiographic norms are available. Metatarsal adduction can be quantified by drawing a line through the heel bisector and noting which toe it intersects. Normally
A
Fig. 1.42 Measurement of the thighfoot angle. (A) Clinical measurement. (B) Normal values with age. (C) Transmalleolar axis and normal values for age. ([B,C] from Staheli LT, Corbett M, Wyss C, King H. Lower-extremity rotational problems in children. J Bone Joint Surg Am. 1985;67(1):43 (Figs. 2E and 2F). Reprinted with permission.)
1 Anatomy and Normal Development in Children 47
B
Fig. 1.42 (continued)
C
Fig. 1.42 (continued)
48 Handbook of Pediatric Orthopedics
A
B Fig. 1.43 (A) Measurement of internal rotation. (B) Measurement of external rotation.
it falls between the second and third toes. Moderate to severe adduction is present when the line falls lateral to the fourth toe. The foot progression angle is the end product of all rotational components in the lower extremity: hip, femur, knee, tibia, and foot. It is the angle formed by the foot (on the average) with the direction of walking (Fig. 1.41). The thigh–foot angle is an approximate clinical measure of tibial torsion. It is assessed with the patient prone and the ankle gently dorsiflexed to a neutral position (Fig. 1.42A). Normal values with age are given in Fig. 1.42B. When the foot is affected by a rotational problem such as clubfoot, metatarsus adductus, spasticity, or contracture, the tibial torsion can be estimated by the transmalleolar axis. This is the angle formed between a line between the two malleoli and the thigh when the patient is prone with the knee bent (Fig. 1.42C). Internal and external rotation of the hip in extension are used to assess the relative contributions of the hip to rotation (Fig. 1.43A,B). Anteversion is likely to be present if internal rotation exceeds 70 degrees and external rotation is less than 20 degrees. Graphs of the normal range with age are given in Fig. 1.43C,D.
1 Anatomy and Normal Development in Children 49
C
D
Fig. 1.43 (continued) Hip rotation is usually measured in extension for assessment of torsion deformities. (C) Normal values for internal rotation with age. (D) Normal values for external rotation with age. ([C,D] from Staheli LT, Corbett M, Wyss C, King H. Lower-extremity rotational problems in children. J Bone Joint Surg Am. 1985;67(1):43 (Figs. 2C,D). Reprinted with permission.)
50 Handbook of Pediatric Orthopedics
Fig. 1.44 Lateral talocalcaneal angle in the standing foot. (From Vanderwilde R, Staheli LT, Chew DE, Malagon V. Measurements on radiographs of the foot in normal infants and children. J Bone Joint Surg Am. 1988;70(3):410 (Fig. 2D). Reprinted with permission.)
Normal Radiographic Measurements of the Pediatric Foot Radiographs are helpful in interpreting pathology in both the unoperated and the postoperative foot. The films should be taken with the patient standing, if possible. Normal values are given for the lateral talocalcaneal
Fig. 1.45 Anteroposterior talocalcaneal angle in the standing foot. (From Vanderwilde R, Staheli LT, Chew DE, Malagon V. Measurements on radiographs of the foot in normal infants and children. J Bone Joint Surg Am. 1988;70(3):409 (Fig. 2A). Reprinted with permission.)
1 Anatomy and Normal Development in Children 51
angle in Fig. 1.44 and the anteroposterior talocalcaneal angle in Fig. 1.45. Note that a decline in the value of both angles is seen with a varus foot (increasing parallelism), and an increase is seen with a valgus foot (increasing divergence).
u Normal Gait in Children Normal gait minimizes energy consumption. Gait deviations occur in response to a patient’s neurologic or mechanical abnormalities. To understand gait by visual or laboratory analysis, it must be broken down into component characteristics.
Definitions 1. Normal walking gait is 60% stance and 40% swing; therefore 20% is spent in double support (both limbs on the ground). 2. Cadence: Number of steps per unit of time 3. Stride: One cycle, including right plus left steps 4. Kinematics: Study of motion 5. Kinetics: Study of forces that produce movement 6. Stance phase: Period when one or both feet are on the ground 7. First rocker: First stage of ankle motion in stance, from heel strike to foot flat; decelerates inertia of body; tibialis anterior contracts eccentrically. 8. Second rocker: Second stage of ankle motion in stance, from foot flat till heel rise; deceleration of tibia to relax quadriceps; soleus contracts eccentrically. 9. Third rocker: Third stage of ankle motion in stance, from heel rise till toe off; accelerates limb; gastrocnemius and soleus contract concentrically. 10. Phases of gait cycle (Figs. 1.46 and 1.47).
Fig. 1.46 Phases of gait cycle. LR, loading response; MS, midstance; TST, terminal stance; PS, preswing; ISW, initial swing; MSW, midswing; TSW, terminal swing.
52 Handbook of Pediatric Orthopedics
Fig. 1.47 Phases of gait cycle with figures. (From Inman VT, Ralston HJ, Todd F. Human Walking. Baltimore: Williams & Wilkins; 1981;26 (Fig. 2.6). Reprinted with permission.)
Normal Parameters of Gait 1. Mature gait: Fully developed by age 7 2. Velocity l l l l
2-year-old: 0.78 m/sec ± 0.2 4-year-old: 1.0 m/sec ± 0.2 6-year-old: 1.1 m/sec ± 0.2 Adult: 1.5 m/sec ± 0.2
3. Cadence l l l
2-year-old: 180 steps/min 4-year old: 160 steps/min Adult: 116 steps/min
4. Hip flexion: Extension 0 to 40 degrees during normal walking 5. Knee flexion: Extension 5 to 60 degrees 6. Ankle: 5 to 20 degrees plantarflexion (see Fig. 1.48 for graphs of normal joint motion during gait)
1 Anatomy and Normal Development in Children 53
Fig. 1.48 Normal joint kinematics at the hip, knee, and ankle. (From Gage JR. The clinical use of kinetics for evaluation of pathological gait in cerebral palsy. J Bone Joint Surg Am. 1994;76(4):626 (Fig. 8, part I). Reprinted with permission.)
Fig. 1.49 Muscle activity during gait.
54 Handbook of Pediatric Orthopedics
Muscle Activity During Gait 1. Muscle activity may be measured by surface electrodes for large muscles or by fine wire electrodes for small muscles. A diagram of normal muscle activity is given in Fig. 1.49. Note that electromyelographic data give muscle activity and intensity but not the force of contraction. 2. Muscle control by phase: a. Heel strike: Gluteus maximus, hamstrings, tibialis anterior b. Loading response: Hamstrings, tibialis anterior, quadriceps, gluteus medius and maximus, adductor magnus c. Midstance: Soleus, quadriceps, gluteus maximus d. Terminal stance: Soleus, gastrocnemius, peroneals, toe flexors e. Preswing: Gastrocnemius, adductor longus, rectus femorus f. Initial swing: Hip flexors, tibialis anterior, toe extensors g. Midswing: Tibialis anterior h. Terminal swing: Hamstrings, quadriceps, tibialis anterior
Bibliography Gage JR. Clinical use of kinetic for evaluation of pathological gait in cerebral palsy. J Bone Joint Surg Am. 1994;76:622–631 Paley J, Talor J, Levin A, Bhave A, Paley D, Herzenberg JE. The multiplier method for prediction of adult height. J Pediatr Orthop. 2004;24(6):732–737 Palmer F, Capute A. Keys to developmental assessment. In McMillan J, ed. Oski’s Pediatrics, 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2006:789
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2 Disorders of Skeletal Growth and Development Selected pediatric orthopedic conditions are summarized here with emphasis on central concepts and parameters. The goal is to provide a working knowledge for treatment. Principles and specific treatment details are covered more extensively in standard texts. Other major topics are covered in Chapters 3 (genetic syndromes), 4 (neuromuscular disorders), and 5 (trauma).
u Lower-Limb Length Inequality Principles 1. Inequalities of lower-limb length of up to 1 to 1.5 cm are within normal variation of the population. 2. Inequalities of less than 2.5 cm do not cause back pain or noticeable limp. 3. Length inequalities are a much less common cause of limp than are joint disorders (contracture, pain) or muscle weakness. 4. The gait disturbance caused by inequality of limb length is usually subtle and consists of pelvic drop and compensatory flexion of the knee of the long limb and equinus of the ankle of the short limb. 5. Most congenital inequalities of limb length behave in a proportionate fashion with growth; that is, the ratio of the short leg to the long leg is constant throughout growth.
Congenital Causes 1. Congenital short femur, proximal focal femoral deficiency, tibial or fibular hemimelia, hemiatrophy, hemihypertrophy. Multiplier method predicts final discrepancy (see Chapter 1). 2. Systemic disorders: Ollier disease, fibrous dysplasia, osteochondromatosis, osteogenesis imperfecta, cerebral palsy.
Acquired Causes 1. Developmental dysplasia of the hip (DDH) (length difference resulting from growth disturbance or osteotomy). 2. Legg–Calve–Perthes disease (may be up to 4 cm if proximal femoral physeal growth slows early. 3. Blount disease 4. Trauma (fracture overlap, growth arrest) 5. Osteomyelitis with physeal arrest 6. Foot deformities
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Measurement 1. Block method: Palpate height of iliac crests in standing patient. Add height to short limb by measured blocks until equal. This is a good screening method to determine whether further, more precise measurements are needed. Although not as precise as radiographic measurements, it does take into account all factors such as contracture and shortening occurring within the foot or pelvis (Fig. 2.1). 2. Tape method: Measure from inferior margin of the anterior superior iliac spine (ASIS) to the medial malleolus. This method can be inaccurate in overweight patients or in those who have had anterior hip surgery and does not include foot discrepancies. 3. Scanogram: Images of both hips, knees, and ankles are taken in one position alongside a radiographic ruler. This method does not account for foot discrepancies or contracture. 4. Computed radiograph: Computes lengths and angles of segments.
Treatment 1. If discrepancy is less than 2.5 cm in a mature person, no treatment is needed. 2. 2.5 to ~4 cm: Leg lift or epiphyseodesis or shortening of corresponding long segment (see section 1).
Fig. 2.1 The standing block test. A block is added under the short limb (B) until the pelvis is palpated to be level (A).
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3. >~4 to 5 cm: Limb lengthening if no contraindications or combination of lengthening and shortening and lift.
Bibliography Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limblength discrepancy. J Bone Joint Surg Am. 2000;82-A(10):1432–1446 Sabharwal S, Zhao C, McKeon JJ, McClemens E, Edgar M, Behrens F. Computed radiographic measurement of limb-length discrepancy: full-length standing an teroposterior radiograph compared with scanogram. J Bone Joint Surg Am. 2006; 88(10):2243–2251
u Developmental Dysplasia of the Hip Principles Developmental dysplasia of the hip (DDH) is caused by forces acting on the hip in utero. The risk is increased by abnormalities of connective tissue. DDH is a spectrum, from hips that are subluxatable to dislocatable (Barlow positive) to dislocated (Ortolani positive). The combined incidence of these groups is 2 to 5 per 1000. All hips should be screened by a knowledgeable examiner at birth and again within the first few months of life. The infant should be made as quiet and comfortable as possible for the examination, using warmth, contact, low light, feeding, or a pacifier. Abnormal physical signs marked with an asterisk (*) should prompt reexamination or ultrasound, with treatment if these are abnormal.
Risk Factors in History The following factors increase the risk of hip dysplasia in descending order and should prompt reexamination or ultrasound: 1. Positive family history of DDH 2. Breech position at the end of gestation (5% are unstable). A breech female should have a screening ultrasound. 3. Large birth weight
Physical Examination for Developmental Hip Dysplasia in the Newborn 1. Appearance at rest: The affected side is more adducted at rest in unilateral cases and may have a deeper or extra high fold proximally. *2. Asymmetric passive abduction: Dislocated hip will lack passive abduction compared with the normal side (Fig. 2.2A). *3. The Barlow test will cause pistoning of the proximal femur if dislocatable (Fig. 2.2B).
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Fig. 2.2 (A) Asymmetric abduction; left side is dysplastic. (B) Barlow test (done on one hip at a time). (C) Ortolani test. *4. The Ortolani test will cause a “clunk” as a dislocated hip is relocated. Examine each hip separately; stabilize the pelvis with the other hand (Fig. 2.2C). Note that the Ortolani and Barlow tests are for translation of the femur. A “click” per se is not a positive test; only 1% of patients with a click have dysplasia. A click may come from the patella or the meniscus of the knee as well as from the fascia lata or a synovial fold in the hip.
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5. Proximal location of greater trochanters is a helpful sign in diagnosing a patient with bilateral irreducible hip dislocations. Also, the femoral head may cause a prominence superiorly. 6. Significant foot deformity or torticollis may increase the risk of hip dysplasia and should prompt a careful examination of the hips.
Evaluation of the Older Child for Developmental Dysplasia of the Hip In the older child with hip dysplasia, the signs progressively change. Reducibility of the hip in the awake patient is lost after about 3 months, and one must rely more on indirect signs: 1. Asymmetric passive abduction 2. Galeazzi test will show thigh shortening on the side that is dislocated (Fig. 2.3A). The pelvis should be kept level during this test. 3. Leg-length discrepancy 4. Trendelenburg gait 5. Palpable femoral head posterior to the acetabulum 6. Nélaton line (an imaginary line between the ASIS and the ischium) should lie superior to the trochanter (Fig. 2.3B). 7. Klisic line between the greater trochanter and the ASIS should project cephalad to the umbilicus (Fig. 2.3C). 8. Increased lumbar lordosis (if bilateral) is due to posterior displacement and mechanical disadvantage of hip abductors.
Ultrasound for Hip Dysplasia Principles The role of ultrasound in diagnosis of dysplasia varies regionally. Its benefit is its ability to show cartilage and other soft tissues, as well as observe stability in response to stress. Interpretation of an ultrasound considers both static and dynamic findings. The ultrasound view is named according to the direction of the transducer— transverse or coronal—and the position of the hip—neutral or flexion. The highest frequency possible (3 to 7 MHz) will give the best resolution, but this must usually be reduced with age to obtain adequate penetration.
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B A
C
Fig. 2.3 (A) Galeazzi sign shows apparent thigh shortening on dysplastic side (right). (B) If dislocated, the greater trochanter will lie proximal to Nélaton line (anterior superior iliac spine to ischium). (C) Klisic line in the normal hip falls above the umbilicus.
Coronal View In this view, the landmarks are similar to those seen on a plain radiograph, when the transducer is in the mid acetabular plane. 1. The stability and gross appearance are the most important features. 2. The following other parameters may be checked (Fig. 2.4):
2 Disorders of Skeletal Growth and Development 61
A
B Fig. 2.4 (A) Ultrasound, coronal view. FhC, femoral head coverage. α angle, acetabular roof line. β angle, slope of labrum. (B) Transverse view. Note the “U” formed by the metaphysis and the acetabulum.
a. Femoral head coverage: The percent of the femoral head medial to the outer line of the ilium. This should be greater than 50%. b. Alpha angle, or acetabular roof line, between the lateral ilium and the bony acetabular roof. It is analogous to the acetabular index and should be greater than 60 degrees. c. Beta angle, or slope of the labrum versus the lateral wall of the ilium. It should be less than 55 degrees, indicating a downward slope of the labrum.
Transverse View In this view the hip is flexed and the transducer is placed posterolaterally in the transverse plane of the body. The combination of echoes from the femoral
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metaphysis and the acetabulum normally form a “U.” When dislocated, the femoral head comes to lie lateral and posterior to the acetabulum, and the U is disrupted.
Radiographic Evaluation of Hip Dysplasia 1. Grades of hip dislocation according to Tonnis indicate the position of the ossific nucleus relative to Perkin vertical line (p) and Hilgenreiner horizontal line (h) (Fig. 2.5). a. b. c. d.
Nucleus medial to Perkin line Nucleus lateral to Perkin line Nucleus at Hilgenreiner line Nucleus above Hilgenreiner line
2. The acetabular index is the angle formed between the Hilgenreiner line and the inner and outer borders of the acetabular roof (Fig. 2.6A, right hip). It is useful in assessing hip development in early years, before the center of the femoral head can be accurately identified. The normal values are shown in Fig. 2.6B. 3. Center edge angle of Wiberg (CEA) a. CEA measures the coverage of the femoral head by the acetabulum (Fig. 2.6A, left hip). Long-term follow-up studies by Wiberg have
Fig. 2.5 Tonnis grades of hip dislocation (p = Perkinsʼ line and h = Hilgenreinerʼs line).
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A
B
Fig. 2.6 (A) Acetabular index measurement (right hip) and measurement of center edge angle of Wiberg (left hip). (B) Acetabular index normal values for age. (Part B from Tonnis D. Normal values of the hip joint for the evaluation of x-rays in children and adults. Clin Orthop Relat Res. 1976;119:41 (Fig. 2). Reprinted with permission.)
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shown a correlation between development of symptoms after maturity and CEA below 20 degrees. b. Normal values Lower limit of normal l 5 to 8 years: 19 degrees l 9 to 12 years: 25 degrees l 13: 26 degrees l Less precise under 5 years
l
Bibliography Tönnis D. Normal values of the hip joint for the evaluation of X-rays in children and adults. Clin Orthop Relat Res. 1976; (119):39–47
Management of Hip Dysplasia The following is a general algorithm for management of dysplasia (Fig. 2.7). Guidelines given may be modified based upon individual factors.
All neonates screened by history and Physical exam ± ultrasound at birth, 2 months, well-child exams Dislocatable or subluxatable (Barlow +)
Risk factors only (history or physical) Breech female
Re-examine in approximately 4 weeks
Re-examine at approximately 2 months
Clinically Improving
Dislocated (Ortolani +) Clinically still unstable
Ultrasound
Exam still equivocal
Exam normal
Abnormal Definitive treatment Late diagnosis < 6 months
Discharge
6-24 months
Pavlik Harness
> 24 months Ultrasound or x-ray in 2 weeks Reduced Check stability and wean by 6-12 weeks
Not Reduced
Closed reduction± traction Unsuccessful Open reduction (medial or anterolateral)
Anterolateral open reduction ±Iliac osteotomy ±Femoral shortening/derotation
Continued monitoring until maturity
Fig. 2.7 General algorithm for management of pediatric hip dysplasia.
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Bibliography Grissom L, Harcke HT, Thacker M. Imaging in the surgical management of developmental dislocation of the hip. Clin Orthop Relat Res. 2008;466(4):791–801 Guille JT, Pizzutillo PD, MacEwen GD. Development dysplasia of the hip from birth to six months. J Am Acad Orthop Surg. 2000;8(4):232–242
u Legg–Calve–Perthes Disease Legg–Calve–Perthes disease (idiopathic avascular necrosis [AVN] of the immature femoral head) is most commonly seen in children aged 4 to 10 years. Five percent of patients develop bilateral involvement, but this is almost always at different times (asynchronous). Synchronous involvement should suggest the possibility of skeletal dysplasia, hypothyroidism, or steroid use.
Symptoms 1. Minimal or no history of trauma history 2. Stiffness 3. Intermittent mild pain or no pain at all
Signs 1. Mild Trendelenburg gait 2. No pain with gentle motion 3. Limitation of extremes of motion, especially abduction and internal rotation
Radiographic Findings 1. Chronological sequence a. Initially may appear normal b. Failure of nucleus to grow versus opposite side c. Subchondral “crescent” sign best seen on lateral view, present in one third of cases d. Fragmentation of nucleus with resorption e. Epiphyseal extrusion outside of acetabulum f. Physeal and metaphyseal irregularities g. Later, reossification and variable remodeling h. Usually loss of epiphyseal and neck height at maturity 2. Staging a. Catterall (Fig. 2.8)
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Fig. 2.8 Catteral classification of Perthes disease as viewed from above. 1) Central anterior involvement of head only 2) Greater central head involvement but intact medial and lateral column 3) Lateral three quarters of femoral head involved with only intact medial column; metaphyseal reaction 4) Whole-head involvement, with metaphyseal reaction and remodeling of epiphysis b. Herring Lateral Pillar Classification predicts flattening during healing (Fig. 2.9). 1) Lateral pillar is intact without radiographic change. 2) Lateral pillar is collapsed, but height is still greater than 50%. 3) Lateral pillar is collapsed to less than 50% of original height.
Fig. 2.9 Herring lateral pillar classification of Perthes.
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3. Prognostic signs a. “Head-at-risk signs” of Catterall 1) Lateral calcification 2) Lateral subluxation 3) Gage sign: Lucency proximal and distal to lateral physis 4) Metaphyseal reaction 5) Horizontal physis (meaning limb is adducted) b. Epiphyseal extrusion (Fig. 2.10) greater than 20% carries poor longterm prognosis. c. Mose sphericity: Deviation of head periphery from a perfect sphere by more than 3 mm on anteroposterior (AP) and lateral radiograph carries poor long-term prognosis. d. Stulberg rating (used after healing) assesses femoral head sphericity and its congruency with the acetabulum. There are five different stages. These have been correlated with long-term outcome (Fig. 2.11). 4. Arthrogram, magnetic resonance imaging (MRI): These are not routinely indicated but may be helpful in selected cases.
Differential Diagnosis 1. Hypothyroidism 2. Multiple epiphyseal dysplasia 3. Spondyloepiphyseal dysplasia
Fig. 2.10 Measurement of epiphyseal extrusion. (From Green NE, Beauchamp RD, Griffin PP. Epiphyseal extrusion as a prognostic index in Legg-Calve-Perthes disease. J Bone Joint Surg Am. 1981;63(6):902 (Fig. 1). Reprinted with permission.)
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Fig. 2.11 Stulberg rating of outcome of healed Perthes. Stages I–II are spherical and congruent and have a low likelihood of degenerative joint disease. Stages III and IV are aspherical and congruous and have a risk of degenerative joint disease by middle age. Stage V is aspherical and incongruous, and there is a risk of degenerative joint disease before 50. Source: Stulberg SD, Cooperman DR, Wallensten R. The natural history of Legg-Calve-Perthes disease. J Bone Joint Surg Am. 1981;63(7):1095–1108.
4. Meyer dysplasia (bilateral synchronous early childhood AVN; better prognosis) 5. Storage disorder (Gaucher disease, mucopolysaccharidoses) 6. AVN following trauma, steroids, sickle cell infarct, DDH treatment
Treatment 1. There is no consensus on a protocol. However, many hips have a poor long-term natural outcome, and some hips appear to be helped by treatment. Containment is indicated if several of the following features are present: a. Head involvement greater than 50% (Catterall 3–4, Herring B or B/C border)
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b. Age over 8 years c. Collapse or extrusion not established yet 2. Containment options a. b. c. d.
Abduction brace or Petrie casts Femoral varus osteotomy Iliac rotational osteotomy or augmentation Combinations of b and c
3. Late options a. b. c. d.
Epiphysiodesis for leg length inequality greater than 2 cm Valgus osteotomy for symptomatic hinge abduction Trochanteric transfer for persistent abductor weakness Epiphyseal osteotomy for femoral incongruity
Bibliography Herring JA, Kim HT, Browne R. Legg-Calve-Perthes disease. Part I: Classification of radiographs with use of the modified lateral pillar and Stulberg classifications. J Bone Joint Surg Am. 2004;86-A(10):2103–2120 Herring JA, Kim HT, Browne R. Legg-Calve-Perthes disease. Part II: Prospective multicenter study of the effect of treatment on outcome. J Bone Joint Surg Am. 2004;86– A(10):2121–2134 Joseph B, Nair NS, Narasimha Rao KL, Mulpuri K, Varghese G. Optimal timing for containment surgery for Perthes disease. J Pediatr Orthop. 2003;23(5):601–606
u Transient Synovitis of the Hip Overview 1. Transient synovitis of the hip is characterized by the acute onset of monarticular hip pain, limp, and restricted hip motion. 2. It is the most common cause of hip pain in children. 3. Child’s age is usually 1 to 4 years, but any age can be affected. 4. This condition must be distinguished from septic arthritis. 5. Gradual but complete resolution over several days to weeks is the norm. 6. Cause is unknown, but it may be immune-mediated.
Diagnosis 1. A diagnosis of exclusion 2. Acute onset of unilateral hip pain in an otherwise healthy child 3. The patient may be afebrile or have a low-grade fever.
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4. Laboratory values are nonspecific and are often within normal limits.
Physical Examination 1. Limp and antalgic gait are common. 2. Most patients can bear weight on the involved extremity with assistance. 3. Hip is held in a flexed, externally rotated position. Restricted range of motion, especially abduction and rotation; slow movement is better. 4. Pain is not as great as with septic arthritis.
Laboratory Tests 1. Laboratory tests are usually nonspecific and within normal limits, but they may help to rule out other diagnoses. 2. Peripheral white blood cell count is normal to slightly elevated. 3. Erythrocyte sedimentation rate averages 20 mm/hour but may be slightly higher. 4. Urinalysis, blood culture, rheumatoid factor, and Lyme titers results are usually within normal limits. 5. Aspiration of joint fluid is not needed if presentation is typical. If done, results are nonspecific.
Imaging 1. Plain films of the hip: AP and lateral views. 2. In transient synovitis they are normal can but help rule out other diagnoses. 3. Ultrasound may be used to assess for effusion and to guide aspiration if infection cannot be ruled out clinically. 4. MRI is needed only in cases of persistent pain.
Differential Diagnosis 1. Septic arthritis 2. Osteomyelitis in the femoral neck or pelvis 3. Tuberculous arthritis 4. Psoas abscess 5. Other muscle infection about the hip 6. Juvenile rheumatoid arthritis 7. Idiopathic chondrolysis 8. Acute rheumatic fever 9. Legg-Calvé-Perthes disease 10. Tumor 11. Sacroiliac joint infection
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Treatment 1. Bed rest at home if diagnosis is clear or in hospital if further workup is needed. 2. Nonsteroidal anti-inflammatory drugs (NSAIDs) 3. Prompt improvement should be seen. 4. Activity as tolerated when clinically improved
Prognosis 1. Good; no clear evidence of increased risk of AVN
Bibliography Dobbs Matthew B. Transient synovitis of the hip. In Morrissy RT, Weinstein SL, eds. Lovell and Winter’s pediatric orthopaedics. Vol 2. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2006:1142–1147. Haueisen DC, Weiner DS, Weiner SD. The characterization of “transient synovitis of the hip” in children. J Pediatr Orthop. 1986;6(1):11–17 Johnson K, Haigh SF, Ehtisham S, Ryder C, Gardner-Medwin J. Childhood idiopathic chondrolysis of the hip: MRI features. Pediatr Radiol. 2003;33(3):194–199 Kocher MS, Mandiga R, Murphy JM, et al. A clinical practice guideline for treatment of septic arthritis in children: efficacy in improving process of care and effect on outcome of septic arthritis of the hip. J Bone Joint Surg Am. 2003;85-A(6):994–999 Kocher MS, Mandiga R, Zurakowski D, Barnewolt C, Kasser JR. Validation of a clinical prediction rule for the differentiation between septic arthritis and transient synovitis of the hip in children. J Bone Joint Surg Am. 2004;86-A(8):1629–1635 Landin LA, Danielsson LG, Wattsgård C. Transient synovitis of the hip. Its incidence, epidemiology and relation to Perthes’ disease. J Bone Joint Surg Br. 1987;69(2):238– 242
u Slipped Capital Femoral Epiphysis Background 1. Incidence: 2 to 10 per 100,000 a. b. c. d.
Higher in males than females Higher in African Americans 20% have bilateral involvement at presentation 20% more bilateral later
2. Etiologic factors a. Obesity b. Trauma: Mild or severe c. Endocrine disorders: Hypothyroidism, hypogonadism, rickets, renal failure
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d. Down syndrome e. Family history f. Radiation
Classification 1. Loder classification l l
Stable: Able to bear weight (even with crutches) Unstable: Unable to bear weight
2. Chronologic l l
Acute: Symptoms of less than 3 weeks’ duration Chronic: Symptoms for 3 weeks or longer
3. Severity l l l l
Grade I: Less than 33% slip of epiphysis on metaphysis Grade II: 33 to 50% slip Grade III: More than 50% slip “Pre-slip”: Symptoms are present in patient at risk, but no observable slip is seen; MRI may be positive.
Clinical Presentation 1. 2. 3. 4. 5. 6.
Age 9 to 14 years, most common Antalgic limp Pain in the thigh, knee, or hip Leg externally rotated during gait and at rest Internal rotation less in flexion than extension Seasonal variation: Highest rates in September, lowest in March because of sunlight and vitamin D cycles 7. Age–weight test: If age is younger than 10 or older than 16 years or weight is less than 50 percentile, suspect nonidiopathic slip and perform an endocrine workup. 8. Height test: If height is below 10th percentile for age, risk of atypical slip is increased increased; perform an endocrine workup.
Radiographic Features 1. Slip is best seen on lateral view. 2. AP view a. Physeal widening, irregularity b. Decreased epiphyseal height
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c. “Klein’s line”: Line on lateral femoral neck with slipped capital femoral epiphysis (SCFE) transects less than 20% of epiphysis in child older than 10 years. d. Chondrolysis–joint space narrowing may be seen before treatment (rare).
Treatment 1. Immediate weight relief (bed rest) 2. Traction for acute slip for comfort or reduction if severe (optional). 3. Fixation in situ a. Single screw is centrally placed within physis. b. Second screw may be used if first is not perfect or if slip is severe. 4. Realignment: Main indication is the patient who is dissatisfied with limb deformity resulting from slip; it is not commonly needed. a. b. c. d.
Open realignment and pinning of acute slip Cuneiform osteotomy just below physis Base of neck osteotomy: some series show high AVN rate. Subtrochanteric osteotomy (Southwick)
5. Prophylactic contralateral pinning a. Cost–benefit studies show prophylactic pinning is justifiable (at the surgeon’s discretion). b. Main indication is a patient in whom diagnosis of late contralateral SCFE may be missed as a result of impaired communication or follow-up. c. Also valid option in patients with SCFE before growth spurt (in girls younger than 10 years, in boys younger than 12 years) d. If triradiate cartilage is closed or in a girl older than 13 years or in a boy older than 14 years, there is a low risk of subsequent slip (~7%).
Complications 1. Chondrolysis: Affects 5% or less and usually improves with time and physical therapy 2. AVN a. b. c. d.
Greater in unstable or acute slips May be focal or complete Some healing is possible in young patients. Some patients can function 10 to 20 years with AVN before salvage is needed.
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Bibliography Carney BT, Weinstein SL, Noble J. Long-term follow-up of slipped capital femoral epiphysis. J Bone Joint Surg Am. 1991;73(5):667–674 Kocher MS, Bishop JA, Hresko MT, Millis MB, Kim YJ, Kasser JR. Prophylactic pinning of the contralateral hip after unilateral slipped capital femoral epiphysis. J Bone Joint Surg Am. 2004;86-A(12):2658–2665 Koenig KM, Thomson JD, Anderson KL, Carney BT. Does skeletal maturity predict sequential contralateral involvement after fixation of slipped capital femoral epiphysis? J Pediatr Orthop. 2007;27(7):796–800 Loder RT, Aronsson DD, Weinstein SL, Breur GJ, Ganz R, Leunig M. Slipped capital femoral epiphysis. Instr Course Lect. 2008;57:473–498 Loder RT, Starnes T, Dikos G. Atypical and typical (idiopathic) slipped capital femoral epiphysis: reconfirmation of the age-weight test and description of the height and age-height tests. J Bone Joint Surg Am. 2006;88(7):1574–1581 Riad J, Bajelidze G, Gabos PG. Bilateral slipped capital femoral epiphysis: predictive factors for contralateral slip. J Pediatr Orthop. 2007;27(4):411–414 Schultz WR, Weinstein JN, Weinstein SL, Smith BG. Prophylactic pinning of the contralateral hip in slipped capital femoral epiphysis: evaluation of long-term outcome for the contralateral hip with use of decision analysis. J Bone Joint Surg Am. 2002;84-A(8):1305–1314 Yildirim Y, Bautista S, Davidson RS. Chondrolysis, osteonecrosis, and slip severity in patients with subsequent contralateral slipped capital femoral epiphysis. J Bone Joint Surg Am. 2008;90(3):485–492
u Developmental Coxa Vara A varus deformity of femoral neck that is usually progressive. Incidence is 1 in 25,000. Thirty percent of cases are bilateral.
Radiographic Features 1. Femoral neck shortened and in varus 2. Inverted “Y” appearance of physis as a result of shear plane through metaphysis 3. Triangular fragment on inferior femoral neck 4. Decreased femoral anteversion 5. Mild acetabular dysplasia
Signs and Symptoms 1. Abnormal gait caused by abductor weakness and leg-length inequality 2. Activity-related hip pain 3. Length inequality if unilateral: Usually less than 2.5 cm
Differential Diagnosis 1. 2. 3. 4.
Cleidocranial dysplasia Metaphyseal dysplasia Morquio syndrome idiopathic
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Fig. 2.12 Hilgenreiner–epiphyseal angle; normal is less than 25 degrees. Note “inverted Y” appearance of physis on involved right hip.
Treatment 1. Observe if Hilgenreiner-epiphyseal angle is less than 45 degrees (Fig. 2.12) 2. Valgus-derotation osteotomy if greater than 45 degrees and progressive and symptomatic or if greater than 60 degrees at diagnosis
Bibliography Weinstein JN, Kuo KN, Millar EA. Congenital coxa vara: a retrospective review. J Pediatr Orthop. 1984;4(1):70–77
u Proximal Focal Femoral Deficiency A spectrum of congenital femoral anomalies including a temporary or permanent discontinuity in the proximal femur
Classification (Aitken) (Fig. 2.13) 1. A: Femur is in continuity but appears discontinuous early. 2. B: Varus/shortening is more extreme, but most ossify later.
Fig. 2.13 Proximal focal femoral deficiency: Aitkin classification.
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3. C: Small acetabulum but no femoral head 4. D: No femoral head or acetabulum Other classification systems exist but are less widely used.
Characteristics 1. 15% are bilateral (most type D). 2. More than 50% have other lower extremity anomalies, most commonly fibular hemimelia. 3. Affected limb is a constant proportion to the length of the normal limb.
Problems 1. 2. 3. 4.
Limb-length inequality (if unilateral) Pelvic–femoral instability Malrotation of lower extremity (flexion–abduction–external rotation) Proximal muscle weakness
Treatment 1. Bilateral a. Patients walk without prostheses if feet are all right. b. Nonfunctional feet may need surgery. c. Extension prostheses may be used when desired to increase height. 2. Unilateral a. Hip abnormalities: Valgus osteotomy and lengthening for types A and B; consider femoropelvic arthrodesis for type D versus containment of thigh segment in a prosthesis. b. Knee: Offer rotationplasty if foot and ankle are strong and positioned distal to the contralateral knee. Syme disarticulation is another option. Both are combined with fusion of anatomic knee to increase lever arm. c. Foot: Prosthesis equalizes length and provides plantigrade foot (Fig. 2.13).
Bibliography Aitken GT. Proximal Femoral Focal Deficiency: Definition, Classification and Management. National Academy of Sciences Symposium, Washington, D.C., 1969 Fowler EG, Hester DM, Oppenheim WL, Setoguchi Y, Zernicke RF. Contrasts in gait mechanics of individuals with proximal femoral focal deficiency: Syme amputation versus Van Nes rotational osteotomy. J Pediatr Orthop. 1999;19(6):720–731 Kalamchi A, Cowell HR, Kim KI. Congenital deficiency of the femur. J Pediatr Orthop. 1985;5(2):129–134 PubMed
2 Disorders of Skeletal Growth and Development 77 Pappas AM. Congenital abnormalities of the femur and related lower extremity malformations: classification and treatment. J Pediatr Orthop. 1983;3(1):45–60
u Bladder Exstrophy A spectrum of anomalies that may involve the bladder, pelvis, intestinal tract, and external genitalia. 1. The most common form is “classic” exstrophy, which involves a widened pelvis with an anterior diastasis, an open bladder, and a complete epi spadias. 2. The mildest form is epispadias, which may have a closed bladder but widened pelvic symphysis. 3. The most pronounced expression of this spectrum is cloacal exstrophy, which usually involves all of the above as well as omphalocele and a lumbosacral neural tube defect. It often includes anomalies of the spine and extremities. 4. The incidence of bladder exstrophy is around 1:25,000 live births. Males are more commonly affected.
Clinical Features 1. Defect in lower abdominal wall 2. Open bladder and urethra 3. In cloacal exstrophy, abdominal wall defect is larger and lower intestinal tract is exposed. 4. A spinal and a neurological examination should be performed. Often in cloacal patients there is lipomeningocele or myelomeningocele. Hip dislocation, foot deformity, or partial sacral agenesis may occur.
Radiographic Features Separation of pubic bones, typically about 4 to 5 cm at birth, and increases steadily with age (Fig. 2.14). The iliac wings are externally rotated and “flattened.” The ischiopubic bones are slightly underdeveloped. The hips themselves rarely show dysplasia.
Treatment 1. The urologist usually performs the reconstruction in several stages, including closure of the bladder and lower abdominal wall soon after birth, followed by epispadias closure at the same time or at a later date. Surgery to achieve continence is commonly performed after the age at which children are normally continent and may consist of bladder neck suspension. 2. Orthopedic surgery of the pelvic deformity is indicated only if it is needed to achieve urologic goals.
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Fig. 2.14 Schematic representation of pelvic differences in classic exstrophy versus normals in the transverse plane.
3. In the neonatal period, pubis may be approximated manually and temporarily held with suturing. 4. In an older child, iliac osteotomy is indicated either anteriorly or posteriorly.
Prognosis The hip function in the untreated patient with classic bladder exstrophy is good. Children walk at a normal age, although they have an increased external foot-progression angle. This becomes less pronounced over time. Adults with exstrophy have an increased incidence of pain in the region of the sacroiliac joints. One natural history study suggests an increased incidence of degenerative disease of the hip in patients with uncorrected exstrophy. Patients with exstrophy are usually fertile.
Bibliography Aadalen RJ, O’Phelan EH, Chisholm TC, McParland FA Jr, Sweetser TH Jr. Exstrophy of the bladder: long-term results of bilateral posterior iliac osteotomies and twostage anatomic repair. Clin Orthop Relat Res. 1980;151(151):193–200 Jani MM, Sponseller PD, Gearhart JP, Barrance PJ, Genda E, Chao EY. The hip in adults with classic bladder exstrophy: a biomechanical analysis. J Pediatr Orthop. 2000; 20(3):296–301 Okubadejo GO, Sponseller PD, Gearhart JP. Complications in orthopedic management of exstrophy. J Pediatr Orthop. 2003;23(4):522–528 Sponseller PD, Bisson LJ, Gearhart JP, Jeffs RD, Magid D, Fishman E. The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg Am. 1995;77(2):177–189 Sponseller PD, Jani MM, Jeffs RD, Gearhart JP. Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am. 2001;83-A(2):184–193
2 Disorders of Skeletal Growth and Development 79
Table 2.1 Comparison of Types of Tibia Vara (by Age) Parameter
Infantile (0–3 yr)
Juvenile (3–10 yr)
Adolescent (11 yr and older)
Pain
No
No
Usually
Site of varus
Proximal tibia only; medial plateau may be tilted or depressed. Femur often in valgus
Tibia
Distal femur and proximal tibia in varus
Risk of bar
Yes
No
Treatment
Observe or brace; hemiepiphyseodesis if mild; osteotomy of femur and tibia as indicated; may need lengthening later
No • Hemiepiphyseodesis if mild, growing • Osteotomy of femur and tibia
u Tibia Vara A focal varus deformity of the proximal tibia resulting from overload causing disordered medial growth. Tibia vara may have onset in the infantile, juvenile, or adolescent period (Table 2.1).
Radiographic Features 1. Normal alignment of the lower extremity is shown in Chapter 1’s Fig. 1.38. 2. In infantile tibia vara, changes involve physeal depression with lucency and beaking of the corresponding metaphysis and epiphysis. These changes have been staged 1 through 7 by Langenskjold (Fig. 2.15).
Fig. 2.15 Langenskjold stages of infantile tibia vara. (From Langenskiold A. Tibia vara. (Osteochondrosis deformans tibiae.) A survey of 23 cases. Acta Chir Scand. 1952;103(1):1–22 (Fig. 5). Reprinted with permission.)
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3. Infants under age 2 years have inadequate ossification to assign Langenskjold stages. As an alternate means of early diagnosis, the metaphyseal– diaphyseal angle may be drawn (Fig. 2.16). An angle of greater than 16 degrees is diagnostic of infantile Blount disease. An angle of less than 11 degrees is rarely seen in patients with Blount disease and rules this diagnosis out. 4. Medial physeal slope greater than 60 degrees may predict recurrent bowing after osteotomy (Fig. 2.17). 5. Medial plateau depression greater than 30 degrees may be seen in neglected infantile tibia vara (Fig. 2.18).
Fig. 2.16 Metaphyseal-diaphyseal angle used to detect tibia vara in very young children. High diagnostic likelihood if angle is greater than 11 to 16 degrees.
Fig. 2.17 Metaphyseal slope is used to predict the risk of recurrent deformity. Values over 60 degrees are at increased risk.
2 Disorders of Skeletal Growth and Development 81
Fig. 2.18 Medial plateau depress ion is unique to infantile tibia vara. If greater than 25 degrees, the medial side may need to be selectively elevated.
6. Mechanical axis deviation quantifies the degree of varus. The axis line is drawn from the center of the hip to the center of the ankle. The degree of varus (or valgus) is quantified as follows (Fig. 2.19).
Differential Diagnosis 1. 2. 3. 4. 5. 6.
Rickets: Many types Achondroplasia Metaphyseal chondrodysplasia (Schmidt) Trauma or infection of medial physis Physiologic bowing Focal fibrocartilaginous dysplasia
Treatment 1. Infantile a. Observe or brace (day versus night) b. Valgus derotation osteotomy if not better by age 4 or Langenskjold stage 4. Hemi-epiphyseodesis is another option.
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Fig. 2.19 Mechanical axis deviation. The location of a straight line between the hip and the ankle is graded in zones as shown.
c. Consider MRI for bar if physis is narrow or medial physeal slope is greater than 60 degrees. If bar is found, resect it or close lateral side of physis. e. Consider tibial plateau elevation if medial depression exceeds 25 degrees. f. Correct femoral valgus deformity if greater than 10 degrees. g. Equalize leg lengths as needed. 2. Adolescent tibia vara a. Correct if deformity exceeds 10 degrees or pain is persistent. b. Lateral hemiepiphysiodesis is an option if deformity is not severe (Mechanical axis deviation 1 to 2) and patient has two or more years of growth remaining. c. Osteotomy of proximal tibia and distal femur as appropriate. d. Equalize leg lengths if discrepancy is greater than 2.5 cm.
2 Disorders of Skeletal Growth and Development 83
Bibliography Feldman MD, Schoenecker PL. Use of the metaphyseal-diaphyseal angle in the evaluation of bowed legs. J Bone Joint Surg Am. 1993;75(11):1602–1609 Gordon JE, King DJ, Luhmann SJ, Dobbs MB, Schoenecker PL. Femoral deformity in tibia vara. J Bone Joint Surg Am. 2006;88(2):380–386 Henderson RC, Kemp GJ Jr, Greene WB. Adolescent tibia vara: alternatives for operative treatment. J Bone Joint Surg Am. 1992;74(3):342–350 Park SS, Gordon JE, Luhmann SJ, Dobbs MB, Schoenecker PL. Outcome of hemiepiphyseal stapling for late-onset tibia vara. J Bone Joint Surg Am. 2005;87(10):2259– 2266
u Other Angular Deformities at the Knee Angular deformities may occur as a result of trauma or metabolic disorders, or they may be idiopathic or physiologic. If deviation from normal alignment is greater than 10 degrees, correction may be indicated in some cases. Treatment may be done by osteotomy and internal or external fixation or by hemiepiphysiodesis. The goal is to restore a horizontal joint line, physiologic angulation (see Chapter 1’s Fig. 1.38) and near-equal limb lengths. Hemiepiphysiodesis may be the simplest method for growing patients. The theory is shown in Fig. 2.20. Prerequisites include (1) predictable growth pattern, (2) sufficient growth remaining to correct defect, and (3) limb lengths close to equal.
Planning 1. Locate the site of deformity—distal femur, proximal tibia, or both—and measure its degree. 2. Using the width of the physis and the desired degree of correction, draw a horizontal line on the figure. 3. Draw a dot at the intersection of this horizontal line with the patient’s tibial or femoral percentile. 4. A vertical line from this point downward indicates the age at which hemiepiphysiodesis should be performed. If done before puberty, it will indicate the length of time before correction is complete. Allow slight overcorrection before removing plates.
Bibliography Bowen JR, Leahey JL, Zhang ZH, MacEwen GD. Partial epiphysiodesis at the knee to correct angular deformity. Clin Orthop Relat Res. 1985; (198):184–190 Stevens PM, Klatt JB. Guided growth for pathological physes: radiographic improvement during realignment. J Pediatr Orthop. 2008;28(6):632–639
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A
B Fig. 2.20 (A) Theory of angular correction by asymmetrical tethering. (B) Angular correction is calculated from metaphyseal width and growth remaining. (From Bowen JR, Leahey JL, Zhang ZH, MacEwen GD. Partial epiphysiodesis at the knee to correct angular deformity. Clin Orthop Relat Res. 1985;198 (Figs. 2 and 3). Reprinted with permission.)
2 Disorders of Skeletal Growth and Development 85
u Patellofemoral Disorders Patellofemoral disorders are common, especially in adolescence. History should include duration of symptoms, recent changes in activity, and inciting factors.
Physical Examination 1. Patient to indicate location of pain 2. Check pain on compression of patella. 3. Apprehension test (guarding with lateral pressure) 4. Assess effusion 5. Note retinacular tightness (reverse tilt) 6. Measure tibial and femoral rotational alignment 7. Assess ligament and meniscal integrity 8. Measure valgus 9. Examine active tracking 10. Observe gait
Differential Diagnosis 1. 2. 3. 4. 5. 6. 7. 8.
Plica Saphenous nerve entrapment Fat pad impingement Patellar osteochondritis Iliotibial band syndrome Patellar malalignment Patellar subluxation Arthrosis
Radiographic Evaluation Radiographs are not needed on initial evaluation of all cases. However, if the problem is recalcitrant, the following studies may help: 1. Merchant view: A “sunrise” view taken at 30 to 45 degrees of knee flexion (Fig. 2.21A). From this, the sulcus angle can be measured, which should be greater than 17 degrees (Fig. 2.21B).
86 Handbook of Pediatric Orthopedics
A
B Fig. 2.21 (A) Merchant view: a sunrise view with knee flexed 30 to 45 degrees. (B) Interpretation. Sulcus angle should be at least -17 degrees. (From Merchant AC, Mercer RL, Jacobsen RH, Cool CR. Roentgenographic analysis of patellofemoral congruence. J Bone Joint Surg Am. 1974;56(7) (Figs. 2 and 5). Reprinted with permission.)
2 Disorders of Skeletal Growth and Development 87
2. The lateral view will show the Insall ratio, which is the length of tendon divided by the length of patella (Fig. 2.22). The normal value is 1.0, and the upper limit of normal is 1.2. 3. Computed tomography may be helpful to assess patellar tilt. It should be done in 20 degrees of flexion. Normal tilt is less than +8 degrees. 4. MRI is an option to assess for osteochondral lesions.
Treatment Treatment of patellofemoral malalignment should start with conservative measures. These may include the following: 1. Stretching of retinaculum, iliotibial band, quadriceps, and hamstrings 2. Strengthening of quadriceps: Low resistance, high repetition, knee not flexed over 45 degrees 3. NSAIDs 4. Orthotics to decrease foot pronation, if present 5. Elastic brace or patellar taping 6. In the resistant case, surgical correction may be offered and may include the following: a. Lateral release (for tilt)
Fig. 2.22 Insall the ratio to evaluate the patella alta: Length of the tendon is divided by the length of the patella. The patella alto is defined as a ratio of greater than 1.2. (From Insall J, Falvo KA, Wise DW. Chondromalacia patellae: a prospective study. J Bone Joint Surg Am. 1976;58(1):2 (Fig. 2). Reprinted with permission.)
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b. Tibial tubercle transfer + vastus medialis oblique advancement (for subluxation) c. Limited debridement (for severe cartilage surface degeneration) d. Optional correction of severe anteversion or torsion
Bibliography Lubowitz JH, Bernardini BJ, Reid JB III. Current concepts review: comprehensive physical examination for instability of the knee. Am J Sports Med. 2008;36(3):577–594
u Discoid Lateral Meniscus Background and Classification 1. Incidence: About 1%; usually lateral 2. Classification: Based on the degree of attachment to the tibial plateau a. Wrisberg ligament type: Absent lateral meniscotibial ligaments. Only ligament of Wrisberg present, attaching meniscus to the posterior cruciate ligament. This type is the most anomalous and unstable type. b. Complete type: Meniscus covers the entire tibial plateau, but ligaments are intact. c. Incomplete type: Stable incomplete discoid meniscus with intact ligaments.
Clinical Signs and Symptoms 1. 2. 3. 4.
“Snapping knee,” typically before the age of 8 years A palpable snap and shift of the tibial plateau during movement Joint pain and swelling occur as the meniscus develops a tear. Feelings of “catching” with knee motion
Imaging 1. Plain radiographs are indicated initially to rule out any bony anomalies. These may show widening of lateral joint space. 2. MRI shows discoid meniscus and any tears. 3. A true discoid meniscus will appear as a continuous bow-tie appearance on three or more consecutive sagittal plane images. In the coronal plane, the discoid meniscus occupies more than one third of the joint diameter at the midpoint. False negatives can occur with the unstable (Wrisberg) type of discoid meniscus, which may maintain a relatively semilunar shape.
2 Disorders of Skeletal Growth and Development 89
Treatment 1. If the patient is asymptomatic and the discoid meniscus is an incidental finding, excision is not recommended. 2. Substantial tears of a stable discoid meniscus may require a resection in which the peripheral rim is left intact (meniscoplasty). 3. In cases in which the meniscus is unstable, repair and contouring may be attempted, but a complete meniscectomy is often required.
Prognosis There is a paucity of long-term follow-up data for both untreated discoid menisci and for partial discoid meniscectomy.
Bibliography Busch MT. Sports medicine in children. In Morrissy RT, Weinstein SL, eds. Lovell and Winter’s Pediatric Orthopaedics. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2006:1309–1310 Dickhaut SC, DeLee JC. The discoid lateral-meniscus syndrome. J Bone Joint Surg Am. 1982;64(7):1068–1073
u Popliteal (Baker) Cyst Background 1. Popliteal cysts are the most common masses found in the popliteal fossa. 2. These synovial-lined ganglion cysts arise from synovial fluid through communication between the knee joint and the bursa between the semimembranosus and medial gastrocnemius muscles. 3. Located along the medial side of the popliteal fossa, posterior to the medial femoral condyle, between the two tendons. 4. Rarely associated with conditions such as meniscal tears, arthritides, and pigmented villonodular synovitis. 5. Prevalence of popliteal cysts in asymptomatic children is around 2%.
Signs and Symptoms 1. Rarely causes symptoms and is usually an incidental discovery 2. May wax and wane in size but should not cause limp or disability 3. However, patients may present with complaints of pain if the cyst ruptures.
90 Handbook of Pediatric Orthopedics
Physical Features Superficial, firm or rubbery, smooth, nontender, slightly mobile and nonpulsatile mass. Most cysts will transilluminate with a handheld light (Fig. 2.23). If so, imaging is unnecessary.
Imaging 1. Differential diagnosis: Lipomas, xanthomas, vascular tumors, or fibrosarcomas 2. History and transillumination are the best ways to differentiate. 3. If in doubt, ultrasound can be used to distinguish between fluid-filled cyst and solid tumors. MRI is another option for those rare cases when clinical confirmation is in doubt.
Treatment Most cysts (85%) disappear without intervention or cause few symptoms (15%). Surgical excision is rarely indicated. Parents should be informed that the cyst may vary in size for a while, but it should eventually regress over the years. Only if the diagnosis is in doubt or if clinical symptoms are persistent should surgery be undertaken.
Fig. 2.23 Transillumination of the popliteal cyst. The cyst picks up light more than surrounding tissues.
2 Disorders of Skeletal Growth and Development 91
Bibliography De Greef I, Molenaers G, Fabry G. Popliteal cysts in children: a retrospective study of 62 cases. Acta Orthop Belg. 1998;64(2):180–183 De Maeseneer M, Debaere C, Desprechins B, Osteaux M. Popliteal cysts in children: prevalence, appearance and associated findings at MR imaging. Pediatr Radiol. 1999;29(8):605–609 Seil R, Rupp S, Jochum P, Schofer O, Mischo B, Kohn D. Prevalence of popliteal cysts in children. A sonographic study and review of the literature. Arch Orthop Trauma Surg. 1999;119(1-2):73–75
u Congenital Dysplasia (Anterolateral Bow/Pseudarthrosis)
of the Tibia
Background and Natural History 1. Congenital dysplasia may be sporadic or associated with neruofibromatosis-1. 2. Etiology is unknown. 3. Anterolateral bowing of distal tibia resembles genu varum, but the apex is more distal and has an anterior component; it is unilateral only. 4. Bowing often progresses to pseudarthrosis, which is usually very difficult to heal with cast, plate, or electrical stimulation. The distal apex contributes to difficulty with fixation. 5. 1–4 cm shortening of the affected side 6. Clinically no pain until fracture (which produces little pain)
Radiographic Appearance (Fig. 2.24) 1. 2. 3. 4.
Narrowing of tibia at bow Narrowing or scalloping of medullary canal Tapering of bone ends May be associated with fibular bowing or pseudarthrosis
Treatment 1. Protect with brace before fracture. 2. Fibular strut if fracture is imminent. 3. If fractured, options include long intramedullary (Williams) rod ± rhBMP-2, Ilizarov treatment, free vascularized fibula, or combinations. 4. Separate treatment for limb-length inequality and valgus ankle may be required.
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A
B
Fig. 2.24 (A) Anteroposterior and (B) lateral radiographs of 3-year-old with neurofibromatosis-1 and congenital tibial dysplasia before the fracture and (C) 2 years after the fracture and rodding.
C
Bibliography Dobbs MB, Rich MM, Gordon JE, Szymanski DA, Schoenecker PL. Use of an intramedullary rod for treatment of congenital pseudarthrosis of the tibia: a long-term follow-up study. J Bone Joint Surg Am. 2004;86-A(6):1186–1197 Dobbs MB, Rich MM, Gordon JE, Szymanski DA, Schoenecker PL. Use of an intramedullary rod for the treatment of congenital pseudarthrosis of the tibia. Surgical technique. J Bone Joint Surg Am. 2005; 87(Part 1, Suppl 1)33–40 Ofluoglu O, Davidson RS, Dormans JP. Prophylactic bypass grafting and long-term bracing in the management of anterolateral bowing of the tibia and neurofibromatosis-1. J Bone Joint Surg Am. 2008;90(10):2126–2134 Richards BS, Oetgen ME, Johnston CE. The use of rhBMP-2 for the treatment of congenital pseudarthrosis of the tibia: a case series. J Bone Joint Surg Am. 2010;92(1):177– 185
u Posteromedial Bow of Tibia Background and Natural History 1. Present at birth. Etiology unknown. Not a prior fracture 2. Bowing resolves with time and growth over 5 to 10 years. 3. No increased risk of fracture
2 Disorders of Skeletal Growth and Development 93
4. Shortening proportionate throughout growth; mean 2 to 5 cm at maturity
Clinical and Radiographic Appearance (Fig. 2.25) 1. Foot appears dorsiflexed and in valgus; limited plantar flexion 2. Leg slightly short at birth. 3. Tibia and fibula have posteromedial bow of distal third with sclerotic cortices.
Treatment 1. Observation with or without stretching and orthosis for foot support 2. Osteotomy rarely necessary for bowing 3. Leg-length equalization as indicated, with lift and surgery near maturity
Bibliography Pappas AM. Congenital posteromedial bowing of the tibia and fibula. J Pediatr Orthop. 1984;4(5):525–531 Shah HH, Doddabasappa SN, Joseph B. Congenital posteromedial bowing of the tibia: a retrospective analysis of growth abnormalities in the leg. J Pediatr Orthop B. 2009;18(3):120–128
Fig. 2.25 Posteromedial bowing of the tibia and fibula in a 1-year-old.
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u Clubfoot Talipes equinovarus may be unilateral or bilateral with equal frequency. Although most cases are idiopathic, other causes or associations should be considered: 1. Neurogenic: Spinal dysraphism, tethered cord, arthrogryposis 2. Connective tissue disorders: Loeys-Dietz syndrome, Larsen syndrome, diastrophic dwarfism, spondyloepiphyseal dysplasia 3. Mechanical: Oligohydramnios, congenital constriction bands 4. Syndromes: Freeman–Sheldon, Pierre-Robin, tibial hemimelia, Mobius
Physical Findings A variable number of these findings may be present: 1. 2. 3. 4. 5. 6.
Medial or posterior crease Curved lateral border Calf atrophy Cavus Equinus Forefoot adduction and supination
The foot may be given a score from 1 to 20 if each positive finding is awarded 1 point (Table 2.2).
Table 2.2 DiMeglio Clubfoot Scoring System Parameter
Points 4 3 2 (degrees) (degrees) (degrees)
1 (degrees)
0 (degrees)
Equinus
45–90
20–45
20–0
0–20 DF
> + 20 DF
Varus
45–90
20–45
20–0
0–20 DF
>20 val
Supination 45–90
20–45
20–0
0–20 pro
>20 pro
Adductus
20–45
20–0
0 >–20 abd
Posterior crease
Yes
No
Medial crease
Yes
No
Cavus
Yes
No
Abnl muscle fcn
Yes
No
45–90
Abbreviations: Abd, abduction; Abnl, abnormal; DF, dosiflexion; fcn, function; pro, pronation; val, valgus. Scoring: very severe = 16–20, severe = 11–16, moderate = 6–10, postural = 1–5.
2 Disorders of Skeletal Growth and Development 95
Radiographs Radiographs are useful mainly if surgery is indicated or in postsurgical follow-up. AP and lateral films should be taken with the foot as plantigrade as possible. Normal values for AP and lateral talocalcaneal angles are given in Chapter 1’s Figs. 1.44 and 1.45. Equinovarus correlates with increasing parallelism of the talus and calcaneus. Cuboid can be neutral or subluxated medially.
Treatment 1. Serial manipulation and long-leg cast treatment (Ponseti method): This treatment produces the most normal foot growth and mobility; it is described in greater detail in Chapter 7. Sequence of correction: a. b. c. d. e.
f. g. h.
Dorsiflexion of first ray Counterpressure on dorsolateral aspect of talus Progressive external rotation and dorsiflexion of hindfoot Achilles’ tenotomy if correction of hindfoot equinus is not complete after correction of other components Maintaining correction after cast removal with foot abduction orthosis. This is a straight or reverse last shoes attached to bar with feet externally rotated 60 degrees. Foot abduction orthosis is worn full-time for 2 months, then nightly for 3 to 5 years. Relapses are salvaged by repeat cast program. Feet with late recurrence are treated by Achilles’ tendon lengthening and transfer of anterior tibialis to third cuneiform.
2. Operative correction, for severe relapses and syndromes, may include the following: a. Lengthening of heel cord and posterior tibialis b. Posteromedial capsulotomies c. Complete subtalar release d. Calcaneocuboid release e. Lateral column shortening
Bibliography Diméglio A, Bensahel H, Souchet P, Mazeau P, Bonnet F. Classification of clubfoot. J Pediatr Orthop B. 1995;4(2):129–136 Dobbs MB, Rudzki JR, Purcell DB, Walton T, Porter KR, Gurnett CA. Factors predictive of outcome after use of the Ponseti method for the treatment of idiopathic clubfeet. J Bone Joint Surg Am. 2004;86-A(1):22–27 Ponseti IV. The Ponseti technique for correction of congenital clubfoot. J Bone Joint Surg Am. 2002;84-A(10):1889–1890, author reply 1890–1891
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u Tarsal Coalition Tarsal coalition is a fibrous, cartilaginous, or bony connection of two or more tarsal bones. It is present in about 3% of the population and is bilateral in half of these. Calcaneonavicular (CN) and talocalcaneal (TC) bars are equally common.
Signs and Symptoms 1. Foot or ankle pain and stiffness occurs at about age 8 to 12 years for CN bar, 12 to 16 for talocalcaneal bar. 2. Limitation of subtalar movement is greater with TC coalition. 3. Pes planus: Variable 4. Peroneal guarding or “spasm”
Radiographs 1. Oblique view of midfoot is diagnostic for CN bar with narrowing, irregularity, or fusion of the space between the two bones (Fig. 2.26A). 2. Lateral view shows pointed projection of calcaneus or “anteater nose” in CN coalition. 3. Harris view shows obliquity and sclerosis of sustentaculum in TC coalition and may show fusion across middle facet. 4. Computed tomography (CT) is the definitive study for a TC coalition. The plane of the tomograms should be the coronal plane of foot with knees flexed and sole flat on gantry. Reconstructions in all planes should be viewed (Fig. 2.26B). 5. CN and TC coalitions may coexist.
Treatment 1. 2. 3. 4.
If discovered incidentally and asymptomatic, observe. Arch support Walking cast for 3 to 6 weeks Bar resection if conservative treatment fails for TC bar less than 50% and no degenerative changes or CN bar of any size 5. Arthrodesis
Bibliography Lemley F, Berlet G, Hill K, Philbin T, Isaac B, Lee T. Current concepts review: Tarsal coalition. Foot Ankle Int. 2006;27(12):1163–1169
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Fig. 2.26 (A) Oblique view of the midfoot is diagnostic for the calcaneonavicular bar with narrowing, irregularity, or fusion of the space between the two bones. (B) Computed tomography is the definitive study for a talocalcaneal coalition. The plane of the tomograms should be the coronal plane of foot with knees flexed and sole flat on gantry. Reconstructions in all planes should be viewed.
u Flatfoot Background and Definition 1. Etiology is a combination of factors including connective tissue properties, muscle tone, and genetics. 2. Achilles contracture increases eversion.
Signs and Symptoms 1. Usually asymptomatic 2. Severe pressure on plantar surface may cause pain.
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3. The natural history is generally benign in all but the most pronounced cases.
Physical Features and Examination 1. Examine elbows, wrists, and knees for evidence of ligamentous laxity. 2. Determine range of hindfoot and forefoot motion to classify as flexible or rigid. 3. Measure ankle dorsiflexion. 4. Observe patient standing on toes, checking for reappearance of the arch as an indicator of structural integrity of the foot during push-off. 5. Inspect skin of sole for signs of pressure concentration over navicular and talar head.
Differential Diagnosis 1. Connective tissue disorders: Marfan, Ehlers Danlos, osteogenesis imperfecta 2. Neurologic disorders: Spinal dysraphism, diplegia 3. Tarsal coalition: Fibrous or bony 4. Congenital malformation: Fibular hemimelia, vertical talus 5. Accessory navicular
Treatment 1. Observation if asymptomatic 2. Soft arch support if symptoms are severe 3. Surgical reconstruction if unresponsive, with structural weakness and pressure concentration
Bibliography Kasser JR. The pediatric foot. In Morrissy RT, Weinstein SL eds. Lovell and Winter’s Pediatric Orthopaedics, 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2008:1257–1329
u Calcaneovalgus Foot Positional deformity in newborns. The foot is dorsiflexed at the ankle and in valgus.
Physical Features Passively, the foot may be plantarflexed to neutral or below, but it does not have a full range of normal plantarflexion. There is no deformity of the tibia.
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Radiographs Not indicated in the typical case because the physical examination is diagnostic. If radiographs are obtained, they show no bony abnormalities other than the dorsiflexed position. This differentiates it from posteromedial bow of the tibia.
Treatment No treatment is necessary. Deformity improves with time. No casting, bracing, or splinting is necessary.
Prognosis Spontaneous resolution is the rule.
Bibliography Kasser JR. The pediatric foot. In Morrissy RT, Weinstein SL eds. Lovell and Winter’s Pediatric Orthopaedics, 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2008: 1257–1329
u Congenital Vertical Talus Essential feature is a dorsolateral dislocation of the talonavicular joint with associated contractures.
Etiology 1. 2. 3. 4.
Idiopathic Myelomeningocele Arthrogryposis Larsen syndrome
Physical Findings 1. 2. 3. 4.
Reversal of arch with plantar convexity,“rocker bottom” Forefoot is dorsiflexed and everted; hindfoot is plantar-flexed. Normal arch is not fully reproducible. Crease in sinus tarsi
Radiographic Findings 1. Talus is almost “vertical” and is in line with tibia 2. Talar axis does not line up with first metatarsal. 3. Diagnostic test: Talus and first metatarsal still do not line up with stress plantarflexion lateral film of foot (Fig. 2.27).
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Fig. 2.27 Diagnostic test for congenital vertical talus. The talus and first metatarsal still do not line up with stress plantarflexion lateral film of foot.
Treatment 1. Plantarflexion casting (into clubfoot position) 2. Percutaneous pinning of talonavicular joint 3. Open reduction via dorsal or plantar approach, with tendon lengthening 4. Subtalar fusion if valgus persists
Bibliography Dobbs MB, Purcell DB, Nunley R, Morcuende JA. Early results of a new method of treatment for idiopathic congenital vertical talus. J Bone Joint Surg Am. 2006;88(6): 1192–1200 Seimon LP. Surgical correction of congenital vertical talus under the age of 2 years. J Pediatr Orthop 1987;7(4):405–411
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u Hallux Valgus Background and Definitions 1. 2. 3. 4.
First metatarsophalangeal (MTP) angle is greater than 15 degrees. Onset before age 10 is termed juvenile hallux valgus. Onset between age 10 and 18 is termed adolescent hallux valgus. Positive family history and ligamentous laxity are common.
Clinical Features 1. 2. 3. 4.
Asymptomatic or pain over medial prominence Tightness of Achilles’ tendon may be present. Pes planus is usually present. Range of toe movement should be measured.
Radiographic Features 1. Standing AP and lateral radiographs are needed. 2. Metatarsophalangeal (MTP) angle greater than 15 degrees is hallux valgus. 3. An intermetatarsal angle (between the 1st and 2nd metatarsals) greater than 10 degrees indicates metatarsus primus varus. 4. The distal metatarsal articular angle (DMAA) is formed between the shaft of the first metatarsal and the line perpendicular to the articular surface of the MTP joint. The DMAA is expected to increase in juvenile or adolescent hallux vagus. 5. MTP joint congruity, relative lengths of the metatarsals, metatarsocuneiform orientation, proximal phalangeal articular angle, and lesser metatarsal orientation should also be assessed.
Treatment 1. Conservative: Shoes should have soft material and a low heel. Arch supports may help with pronation. Night splints have not proven effective. 2. Surgery is indicated only if there is significant pain despite conservative treatment. 3. Metatarsus primus varus (MPV) in adolescent: Lateral hemiepiphyseodesis of first metatarsal base. 4. Deformity of interphalangeal joint: Osteotomy of proximal phalanx. 5. MTP less than 25 degrees: Soft tissue reconstruction. 6. MTP greater than 25 degrees: Correct MPV and reorient joint. 7. Address significant hindfoot valgus with osteotomies. 8. Arthrodesis of the MTP joint is the most reliable way of hallux valgus correction in cerebral palsy.
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Complications Stiffness of the MTP joint, persistent pain, AVN of the metatarsal head, and a high rate of recurrence
Bibliography Aronson J, Nguyen LL, Aronson EA. Early results of the modified Peterson bunion procedure for adolescent hallux valgus. J Pediatr Orthop. 2001;21(1):65–69 Groiso JA. Juvenile hallux valgus: a conservative approach to treatment. J Bone Joint Surg Am 1992;74(9):1367–1374
u Toe Walking Background 1. 2. 3. 4.
Common in toddlers. Commonly caused by shortened Achilles’ tendon. Some eventually adopt normal walking with growth. Persistent toe walking beyond 3 years of age should prompt examination for underlying neuromuscular problems. 5. However, most children have what is termed, by exclusion, idiopathic toe walking. 6. Many patients have a positive family history. 7. May be associated with autism, language disorders.
Signs and Symptoms 1. Painless 2. Occasionally frequent falling
Physical Examination 1. Examine with the child in shorts. 2. Assess for ataxia, muscle weakness, Gower sign. 3. Range of ankle dorsiflexion should be noted, with the knee both flexed and extended. 4. Look for calf pseudohypertrophy. 5. Hamstrings and adductors should be checked for tightness.
Differential Diagnosis 1. 2. 3. 4.
Cerebral palsy Muscular dystrophy Tethered cord syndrome Charcot–Marie–Tooth disease
2 Disorders of Skeletal Growth and Development 103
5. Arthrogryposis 6. Freidreich ataxia
Treatment 1. Physical therapy 2. Casting: Increased ankle dorsiflexion can be achieved by stretching and serial casting. The cast should be changed weekly until the desired ankle range of motion is obtained. After casts are off, continue stretching. 3. Achilles’ tendon lengthening, percutaneous or open.
Bibliography Kalen V, Adler N, Bleck EE. Electromyography of idiopathic toe walking. J Pediatr Orthop. 1986;6(1):31–33
u Macrodactyly Background 1. Macrodactyly is overgrowth of one or several adjacent digits or rays of a hand or foot. 2. It is present at birth, although some cases worsen disproportionately. 3. Growth of the enlarged digits ceases when the patient reaches skeletal maturity.
Classification 1. Incidence is less than 1:10,000. 2. Most cases are idiopathic; others are associated with Klippel–Trenaunay–Weber, neurofibromatosis-1 (NF-1), or Proteus syndrome. 3. Usually unilateral 4. Etiology unknown 5. No genetic risk if not NF-1
Signs and Symptoms 1. Painless in child; premature arthritis in adult 2. Difficulty with shoe wear 3. Impaired push-off (foot) or clumsiness (hand)
Physical Features (Fig. 2.28) 1. Enlargement is greater distally than proximally. The nail is especially enlarged. All tissues are affected.
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Fig. 2.28 Physical features of macrodactyly.
2. Tissues on the plantar or palmar surface of the digit are more enlarged than those on the dorsal side, causing the digit to become hyperextended (dorsiflexed). 3. Central digits are more commonly involved than border digits. 4. Syndactly may co-exist. 5. Metatarsals and carpals are rarely significantly enlarged. 6. If two digits are involved, they grow away from each other. 7. Rarely enlargement is disproportionate. 8. Skin may display hemangioma. 9. Range of interphalangeal motion is decreased.
Tests Genetic testing is available for Proteus syndrome and NF if these are suspected.
Imaging 1. Plain films should be made to help assess and document the extent of overgrowth and the segments involved. 2. Skeletal maturity is often advanced in the enlarged rays. 3. MRI is rarely necessary.
Differential Diagnosis 1. Hemihypertrophy, in which all segments of a limb are uniformly overgrown 2. Acrodactyly, in which overgrowth of all digits is greatest distally 3. Growth hormone excess: Acromegaly
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Treatment 1. Shoe modification if needed 2. Surgery a. Resection of the most enlarged ray, if the width of the hand or foot is greatly increased. b. Phalangectomy may make the length more even if the width is not a problem (if nail loss is acceptable). c. Epiphysiodesis (closure of the growth plates); the correction occurs more gradually with time. 3. Debulking of fat may improve the appearance, especially of the plantar fat hypertrophy. 4. Discretion advised in staging multiple procedures to avoid tissue ische mia.
Bibliography Akinci M, Ay S, Erçetin O. Surgical treatment of macrodactyly in older children and adults. J Hand Surg Am. 2004;29(6):1010–1019 Akinci M, Ay S, Erçetin O, Glutting J. Surgical treatment of macrodactyly in older children and adults. J Hand Surg Am. 2004;29(6):1010–1019 Barsky AJ. Macrodactyly. J Bone Joint Surg Am. 1967;49(7):1255–1266 Dennyson WG, Bear JN, Bhoola KD. Macrodactyly in the foot. J Bone Joint Surg Br. 1977;59:355–359
u Torticollis Malposition of the head and neck with lateral flexion to one side and rotation to the contralateral side. The child holds the ear closer to one shoulder and the chin closer to the other.
Differential Diagnosis 1. 2. 3. 4. 5. 6.
Muscular torticollis resulting from contracture of sternocleidomastoid Atlantoaxial rotatory instability Congenital malformation of upper cervical spine Reflux response (Sandifer syndrome) Ocular or auditory abnormality Brainstem abnormality or tumor
Physical Examination and Findings 1. Measure range of motion to each side 2. Thorough neurologic examination
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3. Palpate for sternocleidomastoid contracture (typically tight on the side of lower ear). 4. Examine eye movements and hearing. 5. Plagiocephaly is a sign of early onset, longer duration.
Imaging 1. Plain films centered on upper cervical spine a. Usually difficult to interpret because of rotation b. Include open mouth AP if possible 2. CT with reconstructions usually necessary 3. MRI if suspect neurologic abnormality or if surgery is planned
Treatment 1. Muscular torticollis: Stretching, possible lengthening 2. Atlantoaxial rotatory instability: Stretching if duration less than 1 week; traction 1 to 3 weeks; fusion if over 1 month
u Idiopathic Scoliosis Idiopathic scoliosis is the most common pediatric spinal deformity. It is transmitted as an autosomal dominant condition with incomplete penetrance. The initial evaluation should rule out other causes and determine maturity, curve size, type, and appropriate treatment. 1. Infantile scoliosis (onset 0–3 years): obtain MRI 2. Juvenile scoliosis (onset 4–9 years): obtain MRI 3. Adolescent scoliosis (onset >9 years)
History 1. 2. 3. 4. 5.
How curve was discovered Presence or absence of significant pain Family history Menarchal status Medical and surgical history
Physical Findings and Examination 1. Record height and weight. 2. Assess trunk and extremities for cutaneous lesions, congenital malformation, connective tissue disorder, or atrophy. 3. Measure pelvic height for inequality of lower-limb length.
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4. Neurologic examination a. Strength, reflexes all extremities b. Abdominal reflex testing 5. Estimate physical maturity (breast appearance, facial or axillary hair) 6. Assess curve a. b. c. d. e.
Shoulder elevation Trunk balance C1 through S1, coronal and sagittal Curve level Intrinsic pelvic deformity Kyphosis and lordosis
Differential Diagnosis (in Absence of Overt Vertebral Malformations) 1. Genetic or connective tissue a. b. c. d. e. f.
Marfan syndrome, Loeys–Dietz Ehlers–Danlos NF Prader–Willi Stickler syndrome Many others
2. Neurologic a. b. c. d. e. f.
Syringomyelia Brainstem or cord tumor Friedreich ataxia Charcot–Marie–Tooth Polio Thoracic level paralysis or dyscoordination of any cause
3. Neoplastic and other a. b. c. d. e.
Tethered cord/occult dysraphism Osteoid osteoma Osteoblastoma Postradiation Spinal cord tumor
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Scoliosis Screening: Forward-Bend Test (Fig. 2.29) 1. Patient stands with feet together, knees straight, palms together. Check shoulders and pelvis for obliquity, and equalize leg lengths with blocks if necessary. Observe sagittal profile for focal kyphosis. 2. Have patient bend slowly all the way over. 3. Check thoracic spine. 4. Check lumbar spine. 5. Scoliometer measurement of trunk asymmetry (Fig. 2.30). Measures the angle of trunk rotation. This is roughly coordinated with Cobb angle. A scoliometer reading of 5 or less is 99% sensitive and 97% specific for curves under 20-degree Cobb angle. The mean Cobb measurement for
Fig. 2.29 Forward bending test for spinal deformity.
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Fig. 2.30 Technique of scoliometer measurement. (From Bunnell WP. An objective criterion for scoliosis screening. J Bone Joint Surg Am. 1984;66(9):1383 (Fig. 3). Reprinted with permission.)
curves 5 degrees by scoliometer is 11 degrees. A scoliometer reading of 7 or less is 88% sensitive and 86% specific for curves under 25 degrees with a mean Cobb of 20 degrees. The scoliometer is adequately sensitive for scoliosis screening: 95% of curves greater than 20 degrees measure 7 degrees or more on the scoliometer.
Bibliography Bunnell WP. Outcome of spinal screening. Spine. (Phila Pa 1976) 1993;18(12):1572– 1580
Radiographic Assessment 1. A radiograph should be made if the physical examination indicates that a curve may require treatment. Posteroanterior (PA) technique minimizes dose to gonads and breast but gives slightly less detail. A lateral film should be ordered only if needed for evaluation of pain or sagittal deformity. 2. Look for other anomalies such as congenital malformation, vertebral erosion, or pedicle widening or thinning. The curve magnitude may be described by the Cobb measurement (Fig. 2.31) and the direction of convexity and levels involved. The interobserver measurement error (Cobb) is around 5 degrees for idiopathic scoliosis and greater than 10 degrees
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Fig. 2.31 Cobb method of measurement. The angle between the upper and lower end vertebrae (EV) in the curve. Risser sign reflects maturation of the ilium. Grade 5 is fused epiphyses. It has a rough correlation with skeletal age and helps to predict the end of skeletal growth. It should not be used in isolation. The stable vertebra (SV) is the lowest vertebra bisected by a vertical line from the center of the sacrum. The apical vertebral (AVT) is measured with respect to the center sacral line.
for congenital scoliosis. Skeletal maturity may be roughly estimated by the Risser sign but should be correlated with physical examination (Tanner) and bone age if needed. 3. Rotation in the adolescent may be estimated by the method of Nash and Moe (Fig. 2.32) or by CT or the Perdriolle method. 4. Rotation may be estimated in the infantile or juvenile patient using the rib–vertebral angle difference (RVAD) of Mehta (Fig. 2.33).
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Fig. 2.32 Rotation may be estimated by the method of Nash and Moe according to the location of the pedicle on the convex side.
Fig. 2.33 Rotation in infantile curves may be estimated by the rib vertebral angle difference of Mehta. The difference is between the angles formed by the two ribs versus the endplate of the apical vertebra.
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Fig. 2.34 King classification of thoracic curve types. 5. Curve types are named for level of the apex: a. Thoracolumbar curves have an apex at or between T12 and L1. 1) Thoracic curves have an apex above this. 2) Lumbar curves have an apex below this. b. Thoracic curves have been classified by King into five types (Fig. 2.34). 1) True double major curves: Both are of equal magnitude and flexibility. 2) False double major curves: Lumbar curve is smaller and more flexible with less prominence. 3) Thoracic curve only; lumbar curve does not cross middle 4) Long thoracic curve; returns to midline at L4 5) Double thoracic curve c. Lenke classification of curve types is more comprehensive and incorporates sagittal characteristics (Fig. 2.35). 1) Main thoracic 2) Double thoracic 3) Double major 4) Triple major 5) Thoracolumbar/lumbar 6) Thoracolumbar/lumbar: Main thoracic
Treatment Guidelines These represent the mainstream of thought, but each case must be managed individually (Fig. 2.36). 1. Infantile scoliosis a. If patient is under age 1 or the curve less than about 25 degrees, observe with follow-up in about 4 months.
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Fig. 2.35 Lenke classification of curve types.
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Fig. 2.36 Idiopathic scoliosis treatment algorithm.
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b. Obtain MRI if curve is greater than about 25 degrees. c. If RVAD is greater than 20 degrees or curve is greater than 35 degrees, consider Mehta casting followed by bracing in younger patients. d. If curve progresses to greater than about 60 degrees, consider growing rods. 2. Juvenile scoliosis a. If curve is greater than 25 degrees, order MRI. b. If curve is greater than 25 degrees, consider full-time brace. c. If curve progresses beyond 50 to 60 degrees, consider growing rods (or stapling) if the patient is younger than 9 years or definitive fusion if over the patient is 10 years or older. 3. Adolescent scoliosis a. Curve over 25 degrees, Risser 0 to 2: Brace full-time or part-time (part-time braces less effective for double curves or those over 35 degrees) b. Risser 3+: Observe or night-brace for relevant patterns c. Discontinue brace when Risser 5 and height gain is less than 1 cm in 6 months d. Curve greater than 50 to 60 degrees: Discuss surgery
u Back Pain in Children Although disabling back pain in children is rare, back pain of some degree is experienced by over 30% of children. History should include the following: 1. 2. 3. 4.
Mechanism of onset Duration Severity (1 to 10) Interference with school, play, or sports
Physical Examination 1. 2. 3. 4.
Neurologic examination Spinal range of motion Location of pain Straight leg raising
Differential Diagnosis 1. Developmental a. Spondylolysis (most common)
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b. Scheuermann kyphosis (second most common) c. Tethered cord 2. Traumatic a. Herniated nucleus pulposus or endplate b. Musculoligamentous strain c. Fracture 3. Infectious a. b. c. d.
Discitis Osteomyelitis Tuberculosis Sacroiliac joint infection
4. Tumor a. Benign bony neoplasm 1) Osteoid osteoma 2) Osteoblastoma 3) Aneurysmal bone cyst 4) Eosinophilic granuloma b. Malignant bony neoplasm 1) Ewing sarcoma 2) Osteogenic sarcoma 3) Leukemia c. Neoplasm: Neural 1) Glioma 2) Epidermoid 3) Neuroblastoma 5. Inflammatory a. Ankylosing spondylitis b. Enteropathic arthritis 6. Extraspinal a. b. c. d.
Neural Intestinal Vascular Psychologic
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Warning Signs of a Serious Underlying Disorder 1. 2. 3. 4.
Neurologic abnormality Repeated interference with function (school, play, sports) Prolonged stiffness Fever
Imaging and Treatment If warning signs are present, aggressive workup including plain radiographs, bone scan, or MRI is indicated. If not, close follow-up with appropriate activity modification followed by an exercise program for abdominal and extensor muscles may be used at first. Further tests and treatment should then be undertaken if needed.
Bibliography Curtis C, d’Hemecourt P. Diagnosis and management of back pain in adolescents. Adolesc Med State Art Rev. 2007;18(1):140–164
u Congenital Scoliosis Introduction 1. Definition: Scoliosis that is due to primary vertebral malformation 2. Types a. Failure of segmentation (Bar): Most deforming (progression 5 degrees or more/year) b. Failure of formation (hemivertebra) 1) Segmented (normal growth plates on either side): Progress up to 2 degrees per year. 2) Semisegmented 3) Unsegmented (nonprogressive) 3. Progression is greatest in first 2 years of life or in adolescent growth spurt.
Associated Findings 1. Spinal dysraphism 2. VATER (vertebral defects [imperforate] anus, tracheoesophageal [fistula], radial and renal [dysplasia]) or VACTERLS association (vertebral anal, cardiac, tracheal, esophageal, renal, limb) malformations 3. Goldenhar syndrome (oculoauriculovertebral dysplasia)
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4. 5. 6. 7. 8.
Arthrogryposis Multiple pterygium syndrome Skeletal dysplasia Klippel–Feil syndrome Sprengel anomaly (congenital scapular elevation)
Imaging 1. 2. 3. 4. 5. 6.
Compare films from infancy Coned AP and oblique films Cardiac auscultation Renal ultrasound or intravenous pyelogram MRI if there is neurologic asymmetry or if surgery is planned Note: Measurement error is greater than in idiopathic scoliosis—10 to 19 degrees.
Treatment 1. Bracing has little or no value. 2. Document progression with films taken in same position every 6 to 12 months. 3. If progressive: a. Acceptable deformity: Fuse in situ (anterior and posterior if wellformed vertebral body) b. Unacceptable deformity 1) Anterior and posterior convex growth arrest if under age 5 2) Osteotomy or excision 3) Careful instrumentation of flexible segment of curve is an option.
u Scheuermann Kyphosis Developmental wedging of three or more adjacent vertebrae over 5 degrees. Incidence is about 5% of general population.
Clinical Features 1. 2. 3. 4.
Thoracic or thoracolumbar kyphosis Pain within curve, usually during growth spurt Mild scoliosis may coexist. Tight hamstrings
Radiographic Findings 1. Wedging of three or more vertebrae
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2. Irregularity of end plate 3. Disk space narrowing 4. Schmorl nodes
Treatment 1. Exercise program: Strengthen abdominal and hip extensor muscles and stretch hamstrings. 2. NSAIDs 3. Bracing of curve if 50 to 70 degrees and skeletal maturity less than Risser 3. 4. Operative correction: Optional for curves greater than 75 degrees if pain persists or deformity is objectionable to patient. a. Posterior column shortening and fusion alone if flexible. b. Anterior release and posterior fusion if rigid.
Bibliography Lowe TG. Scheuermann disease. J Bone Joint Surg Am. 1990;72:940–945
u Spondylolysis and Spondylolisthesis Spondylolisthesis is the most common identifiable cause of back pain in children. The prevalence begins to plateau at around 6% by age 6 years. Etiology is a stress fracture in a susceptible pars interarticularis. The fifth lumbar vertebra is the most commonly involved. Twenty percent of pars defects are unilateral.
Classification 1. 2. 3. 4. 5.
Dysplastic Isthmic (most common) Degenerative Traumatic Pathologic
Risk Factors 1. Risk factors for isthmic spondylolysis a. Positive family history b. Spina bifida occulta of L5 c. Excessive stress 1) Scheuermann kyphosis
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2) Gymnastics 3) Athetosis 4) Football lineman 2. Risk factors for progressive slip a. b. c. d. e.
Preadolescent age Female Dysplastic slip High-grade slip (III or IV) High slip angle
Signs and Symptoms Signs and symptoms most commonly develop in adolescence. 1. Symptoms a. Low back pain: Activity related; worse with extension b. Pain in buttocks or proximal thighs 2. Signs a. Stiff-legged gait b. Limited forward flexion c. Prominent ilia
Fig. 2.37 Measurement of percent slip is performed with respect to a line drawn from the posterior cortex of the sacrum. Note that the reference line from the superior vertebra is parallel to the sacral line, not the L5 cortex. (From Bradford DS. Spondylolysis and spondylolisthesis. In Lonstein JE, Bradford DS, Winter RB, Ogilvie JW, eds. Moe’s Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Philadelphia: W.B. Saunders; 1995;406 (Fig. 19-5A). Reprinted with permission.)
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d. Palpable step-off of spinous process if greater than 25% e. Weakness of ankle or bladder dysfunction (rare)
Plain Radiographic Findings 1. 2. 3. 4. 5.
Pars defect on lateral film Pars defect on oblique film (Scotty dog’s neck) Elongation of pars Vertebral body slippage (see Fig. 2.37 for measurement technique) Relative lumbosacral kyphosis (Fig. 2.38)
Additional Radiographic Studies 1. Bone scan of spine with single-photon emission computed tomography if plain films are negative or need to determine acute versus chronic. 2. CT to visualize or confirm subtle spondylolysis 3. MRI if significant neurologic physical finding exists
Treatment 1. 2. 3. 4. 5.
Activity restriction as indicated Consider brace treatment for healing of acute slip. Strengthen abdominal and extensor muscles. Fusion if severe pain or symptoms persist or if slip is greater than 50% Repair of defect if slip is less than 5 mm and symptomatic and patient is under age 25
Fig. 2.38 Measurement of slip angle (lumbosacral kyphosis). Note that the sacral reference line is drawn perpendicular to the posterior cortex because the sacral end plate may be rounded. (From Bradford DS. Spondylolysis and spondylolisthesis. In Lonstein JE, Bradford DS, Winter RB, Ogilvie JW, eds. Moe’s Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Philadelphia: W.B. Saunders, 1995;406 (Fig. 19-5B). Reprinted with permission.)
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6. Reduction is controversial and usually considered only if slip is high grade and deformity is the primary complaint. 7. Skeletally immature patients should be followed up during growth to watch for progression.
Bibliography Hu SS, Tribus CB, Diab M, Ghanayem AJ. Spondylolisthesis and spondylolysis. Instr Course Lect. 2008;57:431–445
u Osteochondroses Disordered behavior of growing cartilage under load. This may include compressive or tensile loads. Most common age to present is 7 to 12 years.
Classification 1. Spine: Scheuermann kyphosis 2. Upper extremity a. Panner (capitellum) b. Madelung (distal radial physis) 3. Lower extremity a. b. c. d. e. f.
Perthes Osgood–Schlatter (tibial tubercle) Blount (medial tibial physis) Kohler (tarsal navicular) Freiberg (2nd metatarsal head) Sever disease (calcaneal apophysis)
Treatment Cartilage heals and remodels with time. Minimize load. Bracing may help (Blount, Scheuermann). Reconstruct if deformity develops (Perthes, Scheuermann, Blount, Madelung).
Bibliography McKenzie W. Localized disorders of bone. In: Morrissy RT, Weinstein SL, eds. Lovell and Winter’s Pediatric Orthopaedics, 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2005:346–348
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u Musculoskeletal Tumors Most pediatric skeletal tumors are benign. The most common primary malignancies include osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma. Secondary skeletal involvement may occur with leukemia and neuroblastoma and peripheral neuroectodermal tumor.
Symptoms Symptoms that can indicate malignancy include night pain that is not activity related, rapid increase in pain, increasing fatigue, or bruising.
Laboratory Studies 1. Erythrocyte sedimentation rate: a. Mildly elevated for most malignant tumors 2. Complete blood cell count: Abnormal in leukemia and lymphoma
Radiologic Studies 1. Plain films: Location, morphology, and host reaction are most diagnostic features. Locations of most common benign and malignant tumors are shown in Fig. 2.39. 2. Radionuclide scans: Very sensitive for malignant bone and soft tissue tumors; may be negative in eosinophilic granuloma 3. CT: Best when bony changes need to be better defined 4. MRI: Does not show bony detail well but does show soft tissue and intramedullary detail well
Tumor Types 1. Benign a. Eosinophilic granuloma: Reticuloendothelial lesion usually centrally located in one or several bones; poorly or well circumscribed. Usually waxes and wanes spontaneously. b. Osteoid osteoma: Painful nidus surrounded by sclerosis; usually found in patients aged 6 to 25 years. c. Osteochondroma (osteocartilaginous exostosis), metaphyseal, solitary, or multiple; growth ceases at maturity. d. Chondromyxoid fibroma: Eccentric local lesion, usually in lower extremity progressively enlarging. Patients aged 10 to 25 years. e. Chondroblastoma epiphyseal tumor of adolescence; lucent with foci of internal calcification.
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Fig. 2.39 Sites of musculoskeletal tumors in skeletally immature and skeletally mature children. See text for explanation of abbreviations. f. Unicameral bone cyst: Central lucent metaphyseal lesion usually of proximal humerus or femur; expands and thins cortex; resolves at maturity. g. Nonossifying fibroma: Eccentric intracortical deficit in metaphysic; resolves by maturity. h. Adamantinoma (adam): Sclerotic anterior cortical deficit, usually of tibia. i. Enchondroma (ench) central lucent defect with internal calcification.
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j. Giant cell tumor: Lucent epimetaphyseal tumor just after maturity. k. Aneurysmal bone cyst (ABC): expansile metaphyseal lesion of late adolescence that destroys cortex but leaves thin shell. 2. Malignant a. Fibrosarcoma b. Osteosarcoma: Most common primarily malignant bone tumor, metaphyseal; located in fastest growing regions c. Chondrosarcoma: Central or peripheral, expansile tumor of young adults d. Ewing sarcoma: Small cell tumor of diaphysis; patients aged 5 to 15; usually lasts with extensive periosteal reaction
Tumors Common to Specific Locations in Children 1. Long bones (Fig. 2.39) 2. Spine a. Posterior elements 1. Aneurysmal bone cyst 2. Osteoid osteoma 3. Osteoblastoma b. Vertebral body 1. Histiocytosis 2. Hemangioma 3. Osteosarcoma 4. Ewing sarcoma 5. Chordoma 3. Ribs a. b. c. d.
Fibrous dysplasia Ewing sarcoma Chondrosarcoma Metastasis
4. Pelvis a. b. c. d. e.
Ewing sarcoma Fibrous dysplasia Aneurysmal bone cyst Osteoblastoma Eosinophilic granuloma
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f. Leukemia g. Osteosarcoma 5. Scapula a. Ewing sarcoma b. Osteoblastoma c. Aneurysmal bone cyst
Staging 1. Malignant tumors (Table 2.3) 2. Benign tumors a. Latent b. Active c. Aggressive; May expand into soft tissues or metastasize (Table 2.4)
u Musculoskeletal Problems in Hemophilia Hemophilia A (factor VIII) and B (factor IX deficiency) are the two most common bleeding disorders, followed by von Willebrand disease. Factor levels below 5% of normal level indicates risk of serious bleeding. These conditions should be jointly managed by hematology and orthopedic specialists (Table 2.5).
Treatment of Acute Hemarthropathy 1. 2. 3. 4.
Factor replacement 50% every 48 hours × 6 days Aspiration Immobilization Rehabilitation
Table 2.3 Malignant Tumors Surgical Stage IA IIA III
Surgical Grade (G)
Site (T)
Metastases (M)
Low (G1)
Intracompartmental (T1)
M0 B
Low (G1)
Extracompartmental (T2)
M0
High (G2)
Intracompartmental (T1)
M0
High (G2)
Extracompartmental (T2)
M0
Any
Any T
Source: Enneking WJ. Musculoskeletal Tumor Surgery. New York: Churchill Livingstone, 1983.
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Table 2.4 Common Bone Tumors Type of Tumor
Abbreviation
Name
EG
Eosinophilic granuloma
OO/OB
Osteoid osteoma/osteoblastoma
OCE
Osteochondroma
CMF
Chondromyxofibroma
CB
Chondroblastoma
UBC
Unicameral bone cyst
Benign
NOF/FCD
Nonossifying fibroma/fibrous cortical defect
Adam
Adamantinoma
Ench
Enchondroma
GCT
Giant cell tumor
ABC
Aneurysmal bone cyst
FS
Fibrosarcoma
OS
Osteosarcoma
CS
Chondrosarcoma
MFH
Malignant fibrous histiocytoma
POS
Parosteal osteosarcoma
Met
Metastasis
SS
Synovial sarcoma
RMS
Rhabdomyosarcoma
Malignant
Treatment of Subacute Hemarthropathy 1. Factor replacement 30% X 2–6 weeks. 2. Strengthen and increase ROM. 3. Consider synovectomy if not resolved
Table 2.5 Factor Doses and Kinetics Factor
% Rise After l Unit/kg Dose
Approximate Half-life (h)
VIII
2
12
IX
1
24
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Control of Bleeding after Fracture 1. Factor replacement 50% X 1–2 days. 2. Factor replacement 30% X 1 week.
Radiographic Evaluation Arnold Classification 1. 2. 3. 4. 5.
Soft tissue swelling Osteopenia, epiphyseal overgrowth Subchondral cysts; wide or square contours Irregular joint space Absent joint space
Bibliography Heyworth BE, Su EP, Figgie MP, Acharya SS, Sculco TP. Orthopedic management of hemophilia. Am J Orthop. 2005;34(10):479–486
u Musculoskeletal Infections Evaluation/Workup Obtain all necessary cultures before starting antibiotics. 1. 2. 3. 4. 5.
Blood cultures in all, if possible Spinal tap if indicated Aspiration of bone, joint, or abscess MRI if multiple tissues involved Bone scan if location difficult on physical examination
Differential Diagnosis 1. Transient synovitis 2. Postinfectious arthritis 3. Juvenile rheumatoid arthritis
Table 2.6 Empiric Antibiotic Selection for Skeletal Infection Age (mo)
Antibiotic
4
Clindamycin; or vancomycin +Rifampin for severe infections
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Table 2.7 Organisms Causing Osteomyelitis Organism
%
Staphylococcus aureus, MS
22
Staphylococcus aureus, MR
22
Staphylococcus epidermidis
5
Grade A β-hemolytic streptococcus
4
Pseudomonas aeruginosa
4
Enterobacter cloacae
1
Kingella kingae
1
Streptococcus pneumoniae, Escherichia coli, enterococcus, Candida spp.
each 50 degrees) 3. Preoperative MRI or computed tomography/myelogram on all dystrophic curves 4. Rule out sarcoma if unexplained pain or localized growth occurs
u Ehlers–Danlos Syndromes This group of connective tissue abnormalities has at least 11 different subtypes. Beighton later suggested six descriptive types (classic, hypermobility, vascular, scoliotic, arthrochalasis, dermatosparaxis) and five unspecified types. Many other patients do not fit precisely into one of the groups. Most are disorders of collagen types 1, 5, or its processing enzymes. They are listed in Table 3.2.
3 Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics 155
Table 3.2 Types of Ehlers-Danlos Syndrome Names
Type Genetics
Skeletal Manifestations Dislocations Joint Scoliosis Laxity
Classic: gravis 1
Other Problems
AD
+
+
+
Aneurysms, viscus rupture, hernias
Classic: mitis
2
AD
-
+/−
-
—–
Benign hypermobile
3
AD
+
+
-
Mitral valve prolapse
Vascular
4
AD/AR
+
Fingers -
Aneurysms, spontaneous rupture
Unspecified X-linked
5
X
-
-
-
Intramuscular hemorrhage, “floppy baby”
Ocular– scoliotic
6
AR
+
+
++
Ocular complications
Arthrochalasis 7 multiplex
AR
+
+
+
Short stature
Unspecified: 8 periodontosis
AD
-
+/−
-
Necrobiosis of skin; periodontosis
Unspecified: 9 occipital horn
X
+
+
-
Occipital horns, skeletal dysplasia
Hands
-
Platelet defect
Unspecified: platelet dysfunction
10
AR
-
Unspecified: familial laxity
11
AD
Petallae, hips
+
Abbreviations: AD, autosomal dominant; AR, autosomal recessive.
u Osteogenesis Imperfecta Osteogenesis imperfecta (OI) comprises a group of disorders of type I collagen causing bone fragility and in some cases blue sclerae, hearing loss, and abnormal dentin. The osseous fragility tends to improve after puberty.
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Sillence Types l
Type I a. b. c. d.
l
Variable osseous fragility (minimal through moderately severe) Blue sclerae (at all ages) Early hearing loss Autosomal dominant
Type II (lethal perinatal OI) a. Extremely severe osseous fragility, with stillbirth or neonatal death b. Subgroup A—radiographs show broad, crumpled long bones and broad ribs with continuous beading. Autosomal dominant or new mutation c. Subgroup B—radiographs show broad crumpled long bones, ribs show discontinuous beading or are not beaded. Autosomal recessive d. Subgroup C—radiographs show thin, fractured long bones and thin, beaded ribs. Autosomal recessive (?)
l
Type III a. Autosomal recessive b. Fractures at birth, then progressive deformity c. Normal sclerae and hearing
l
Type IV a. b. c. d.
l
Moderate osseous fragility Normal sclerae (blue in infancy) Variable deformity of long bones and spine Autosomal dominant
Note: The value of opalescent dentin for subcategorization of OI is uncertain.
Additional Types (After Sillence) l
Type V a. b. c. d.
Hyperplastic callus Radial head dislocation Moderate fracture rate Normal type I collagen
3 Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics 157 l
Type VI a. b. c. d.
l
Type VII a. b. c. d.
l
Moderate osseous fragility “Fish-scale” pattern of lamellae on histology Excessive osteoid deposition Normal type I collagen
Rhizomelic short stature Normal type I collagen CRTAP (cartilage-associated protein) defect Reported only in Canadian First Nations Community
Type VII a. b. c. d.
Short stature and normal sclerae Bulbous metaphyses Extreme osteopenia Normal type I collagen; attributable to defect in leprecan, which hydroxylates proline #986 of COL 1A1
Bruck Syndrome 1. OI with multiple congenital contractures 2. Genetic defect in bone-specific telopeptide lysl hydroxylase 3. Contractures of knees, feet and ankles, elbows
u Mucopolysaccharidoses Mucopolysaccharidoses are recessive disorders of glycosaminoglycan (mucopolysaccharide) storage, all autosomal recessive. They have delayed appearance of signs and symptoms corresponding to the accumulation of storage products. Most are progressive. Table 3.3 describes their features.
u Malformations of the Hand and Foot Syndactyly 1. Terminology a. Extent: Partial or complete b. Simple: Skin only c. Complex: Synostosis
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Table 3.3 The Mucopolysaccharidoses Number Name
Genetics Enzyme Defect
I-H
Hurler
AR
µ-L-iduronidase
Diagnosis at 1–3 yr; corneal clouding, MR, kyphoscoliosis. Some amelioration with enzyme replacement, BMT, gene therapy
I-S
Scheie
AR
µ-L-iduronidase
Corneal clouding, aortic abnormality, normal intelligence, longer survival
II
Hunter
XR
Iduronate sulfate sulfatase
Clear cornea, mild MR, ± kyphosis
III
SanFillipo A,B,C,D
AR
IV-A
Morquio-A
AR
B galactosidase - 6-sulfate sulfatase (increased urinary keratan sulfate)
Short trunk, odontoid hypoplasia with cervical instability, flame-shaped vertebrae, kyphosis (TL)
IV-B
Morquio-B
AR
B galactosidase
Milder form
VI
Maroteaux– AR Lamy
Arylsulfatase B
Corneal clouding, normal intelligence, ± cervical stenosis, ± TL kyphosis
VII
Sly
B-glucuronidase
May have epiphyseal dysplasia
AR
Clinical Features
Dementia, seizures
Abbreviations: AR, autosomal recessive; BMT, bone marrow transplant; MR, mental retardation; TL, thoracolumbar; XR, X-linked recessive.
d. Polysyndactyly: Hidden duplicated skeletal structures e. May be associated with Apert syndrome, Saethre–Chotzen, Poland, or other syndromes 2. Isolated syndactyly (five types) a. b. c. d.
Long-ring syndactyly is most common. Look for duplicated phalanges, abnormalities of metacarpals and tarsals. Autosomal dominant Minimal risk of associated anomalies
3. Poland syndrome a. Simple syndactyly of variable number of fingers b. Short fingers (absent or hypoplastic middle phalanges) c. Absent sternocostal head of pectoralis major
3 Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics 159
4. Acrocephalosyndactylies a. Apert syndrome 1) Complete complex syndactyly D2–4 with common nail, progressive interphalangeal synostosis of hands and feet. Medial deviation of great toe and tarsal synostosis 2) Craniosynostosis 3) Occasional cervical fusions, usually without deformity b. Crouzon syndrome 1) Craniosynostosis 2) Calcaneocuboid coalition, C-spine fusion c. Many others: Saethre-Chotzen, Carpenter, etc. 5. Congenital constriction bands (Streeter’s bands) a. b. c. d. e.
Distal (acral) syndactyly Thumb rarely involved Cutaneous rings or amputations May have distal paresis or deformity (clubfoot) No known mendelian basis but genetic contribution possible
Polydactyly 1. Ulnar (postaxial): Frequently isolated, especially in African Americans 2. Radial (preaxial): More frequently associated with syndromes, especially radial ray defects 3. Radial clubhand: This is a spectrum that includes hypoplasia to complete absence of preaxial parts. It may be isolated or associated with the following: a. Blood dyscrasias 1) Fanconi: Anemia to progressive pancytopenia, not present at birth; about a third with renal anomalies; often fatal 2) TAR (thrombocytopenia, absent radii) syndrome; neonatal thrombocytopenia, usually improves with time; frequent knee anomalies b. Congenital heart defects 1) Holt–Oram syndrome: Variable cardiac and preaxial deficiency; most commonly atrial septal defect and hypoplastic thumb c. Craniofacial anomalies (Nager syndrome) d. Congenital scoliosis 1) VATER, Goldenhar syndrome (oculo-auriculo-vertebral dysplasia) 2) Klippel–Feil syndrome
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4. Implications a. Examine previous chest and abdominal films for vertebral anomalies, or take new ones b. Evaluate face, jaw, palate c. Do complete blood and platelet counts d. Ask about feeding (esophageal abnormalities) e. Listen to heart, possibly echo f. Evaluate genitourinary system: Urinalysis, possibly echo g. Chromosome analysis if multiple anomalies found outside of the particular syndrome
Ulnar Clubhand 1. Usually a mild dysgenesis; few frequent associations 2. May be seen with Cornelia de Lange syndrome
Amputated Limbs 1. Single: Usually an isolated anomaly but may be associated with idiopathic scoliosis 2. Congenital ring constriction syndrome a. Nongenetic, variable, rings with grooves in skin, occasionally with lymphatic or vascular impairment b. Transverse amputation with proximal limb normal c. Syndactyly (distal with proximal fenestrations) d. Clubfeet e. Craniofacial defects
u Syndromes with Predominant Spinal Deformity VA(C)TER(LS) 1. A syndrome or association of unknown etiology, characterized by l l l l l
l l
Vertebral anomalies Anorectal atresia Cardiac anomalies TE (tracheosesophageal) fistula Renal and radial anomalies (renal atresia, duplication; radial clubhand or preaxial upper-limb hypoplasia) Lower-limb abnormalities (duplicated hallux or other anomalies) Single umbilical artery
3 Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics 161
2. Clinical implications: In patient seen for vertebral anomaly, search for other abnormalities. Obtain renal ultrasound or MRI.
22Q Deletion Syndrome 1. Common chromosomal deletion syndrome encompassing DiGeorge syndrome, velocardiofacial syndrome, conotruncofacial syndrome, and others 2. Key anomalies include skeletal, palatal, cardiac, and immunologic. 3. Spinal features: Platybasia, occipitalization of the atlas, atlas arch defects, block vertebrae, stenosis, up-turned (swoosh) C2 lamina, thoracolumbar anomalies 4. Other skeletal anomalies: Equinovarus feet, polydactyly, rib anomalies
Wildervanck Syndrome Deafness, Klippel-Feil anomaly, abducens palsy, Chiari malformation
Goldenhar Syndrome See preceding sections.
u Syndromes Caused by Teratogens Fetal Alcohol Syndrome 1. Growth disturbance (of both length and weight) through childhood 2. Central nervous system dysfunction and decreased head size; learning deficit/attention deficit disorder/mental retardation 3. Dysmorphic face (mild): Small eyes, flat philtrum, thin upper lip 4. Orthopedic features a. Contractures (elbows, metaphalangeal and interphalangeal joints) b. Miscellaneous synostoses c. Hip dislocations, clubfeet d. Congenital cervical fusion (C2-C3 = most)
Fetal Hydantoin Syndrome (from Maternal Use of Phenytoin) 1. 2. 3. 4.
Growth retardation: Mild Mental retardation: Mild Face: Hypertelorism, cleft lip Hands: Hypoplasia, absence of phalanges, mostly distal
Warfarin Fetal bleeding and teratogenesis
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u Chromosome Abnormalities Down Syndrome 1. Trisomy, mosaicism, or translocation of chromosome 21 2. Major findings a. Mental retardation, variable b. Congenital heart defects: arteriovenous communis, ventriculoseptal defect c. Gastrointestinal anomalies d. Short stature e. Leukemia (1%), seizures, diabetes, hypothyroidism: Less frequent f. Orthopedic 1) Delayed walking (1½ to 5 years of age) 2) Ligamentous laxity 3) C1-C2 or occiput: C1 laxity: Radiographs at about age 4 and yearly if atlanto-dens interval is greater than 5 mm. Fuse if signs of myelopathy exist. 4) Scoliosis, idiopathic-like 5) Hip dislocations: Acute, subacute, or habitual 6) Slipped capital femoral epiphysis 7) Perthes disease 8) Patellar subluxation or dislocation 9) Metatarsus adductus or hallux valgus
Turner Syndrome 1. Monosomy X 2. Major findings a. b. c. d. e. f.
Low birth weight and persistent growth retardation Normal intelligence Low hairline and webbed neck Renal and cardiac anomalies, coarctation Absent or hypoplastic gonads Orthopedic 1) Genu and cubitus valgus 2) Scoliosis, idiopathic
Noonan Syndrome 1. Turner-like phenotype but normal chromosomes. Defect in PTPN11 in some cases
3 Skeletal Syndromes and Systemic Disorders in Pediatric Orthopedics 163
2. Major findings a. b. c. d. e.
Mental retardation Hypertelorism, ptosis, downward-slanting eyes Increased severity of scoliosis, end-plate changes Pulmonary stenosis common Thrombocytopenia in some cases
Klinefelter Syndrome 1. Genetics: 1. 47 XXY 2. Clinical features a. b. c. d.
Asthenic habitus, long legs Failure of secondary sexual development Scoliosis Proximal radioulnar synostosis
“Cri-du-Chat’’ Syndrome 1. Genetics: 5P 2. Clinical findings a. Profound mental retardation b. Multiple hand and foot anomalies c. Scoliosis, congenital
Bibliography Bethem D, Winter RB, Lutter L, et al. Spinal disorders of dwarfism: review of the literature and report of eighty cases. J Bone Joint Surg Am. 1981;63(9):1412–1425 Goldberg MJ. The Dysmorphic Child—An Orthopaedic Perspective. New York: Raven Press; 1987 McKusick V. Online Mendelian Inheritance in Man. Accessible online through National Library of Medicine Entrez-Pub Med site Morrissey RT. Genetic aspects of ortho conditions. In Lovell & Winter’s Pediatric Orthopaedics, 6th ed. Philadelphia: J.B. Lippincott; 2006 Morrissey RT. The skeletal dysplasia. In Lovell & Winter's Pediatric Orthopaedics, 6th ed. Philadelphia: J.B. Lippincott; 2006 Shirley ED, Sponseller PD. Marfan syndrome. J Am Acad Orthop Surg. 2009;17(9):572– 581
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4 Neuromuscular Disorders in Pediatric Orthopedics This chapter summarizes neuromuscular diseases that affect the skeleton as a result of pathologic conditions of the spinal cord or peripheral nervous system. These diseases may present a fully developed picture or show early subtle findings, such as a mild deviation of gait. The tables in this chapter organize the neuromuscular diseases according to the location of pathology reference.
u Evaluation The elements of evaluation of neuromuscular disease include the following: 1. History: Prenatal, birth (gestation, weight, apgar scores), developmental (milestones), family 2. Physical examination a. b. c. d.
Motor strength, tone Deep tendon reflexes Cranial nerves Cerebellar signs
3. Serum creatine phosphokinase (CPK) a. Elevation directly related to amount of muscle necrosis or membrane disorder. Abnormal in Duchenne and Becker muscular dystrophies (>20 times normal in childhood and early teens), myopathies, or myositis b. Minimal to mild elevation in other dystrophies 4. Immunocytochemistry: Directly differentiates Duchenne from Becker dystrophies and other conditions. Requires small amount of muscle tissue 5. DNA mutation analysis: Requires small amount of blood or amniotic fluid, allows prenatal diagnosis 6. Electromyelography (EMG): Distinguishes myophatic from neuropathic weakness a. Myopathic process: Polyphasic low-voltage signal; fibrillations and sharpwaves b. Neuropathic process: Initially, brief biphasic low-voltage fibrillation c. Chronic process: Prolonged polyphasic fibrillation, increased amplitude
4 Neuromuscular Disorders in Pediatric Orthopedics 165
7. Nerve conduction studies a. Abnormally slowed conduction velocities in conditions involving peripheral nerves only b. Normal conduction velocities in spinal muscular atrophy c. Normal conduction velocity (>49 m/s in arms, >39 m/s in legs) for patients over 5 years of age. Younger children have slower conduction velocity. 8. Muscle biopsy: Biopsy minimally involved muscle in chronic conditions and severely involved muscle in acute conditions. Vastus lateralis used for proximal myopathy, should be done only in centers capable of doing special histochemistry a. b. c. d. e.
Type I fibers: Oxidative metabolism, slow twitch Type II fibers: Anaerobic metabolism, fast twitch Myopathic process Necrosis, phagocytosis, and inflammation Irregularly sized fibers Type I predominance less than 50% Neuropathic process Small group atrophy Fiber-type grouping, angular fibers Type II predominance greater than 80% Electron microscopy (glutaraldehyde) is used to differentiate architectual changes within the congenital myopathies
9. Nerve biopsy a. Sural nerve most commonly biopsied b. Guillain Barré syndrome: Mononuclear infiltrates and focal acute demyelination c. Hypertrophic neuropathies: Nerve fiber loss interstitial fibrosis and “onion bulb” formation 10. Electrocardiogram a. Abnormal in Duchenne muscular dystrophy (sinus tachycardia and right ventricular hypertrophy), Friedreich ataxia, and myotonic dystrophy
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u Cerebral Palsy Definition Static brain injury or lesion in prenatal, perinatal, or postnatal period (up to 2 years)
Causes Prematurity, infection, hypoxia, ischemia, embolus, trauma, brain malformation
Physiologic Types Pyramidal (spastic), extrapyramidal (dystonic or athetoid), mixed
Anatomic Types 1. Diplegic: Usually premature. Pyramidal tracts involved; magnetic resonance imaging (MRI) shows periventricular leukomalacia; cognition preserved; legs affected, most distally; equinovalgus feet most common; hips rarely subluxate; scoliosis is rare. 2. Hemiplegic: Focal cortical lesion; risk of seizure; cognition preserved; ipsilateral arm and leg involved. Equinovarus foot is most common; hip and spine involvement is rare. Limb shortening is usually minor. 3. Totally involved (quadriplegic): Usually diffuse cortical problem. Cognition may be affected; problems with spine and trunk balance; respiration and swallowing function affected. Risk of seizure. Hip subluxation, scoliosis, and limb deformities are common. 4. Other types: Monoplegia, asymmetric diplegia
Function Gross Motor Functional Classification System (Fig. 4.1) 1. I: Walks without restrictions, but speed and coordination are impaired. 2. II: Walks in and outdoors and climbs stairs using railing; difficulty with uneven surfaces 3. III: Uses crutches to walk. Propels own manual wheelchair 4. IV: Walks short distance with walker; uses wheelchair more 5. V: Trunk and all extremities are involved. No independent mobility
Hip Subluxation Rating Reimer migration index (MI) is the percentage of femoral head lateral to Perkin line (A/B below); quantitates MI (Fig. 4.2).
Treatment 1. Physical therapy 2. Mobility aids (orthoses, crutches, walker, wheelchair)
4 Neuromuscular Disorders in Pediatric Orthopedics 167
Fig. 4.1 Gross motor functional classification system. (From Graham HK. Classifying cerebral palsy. J Pediatr Orthop. 2005;25(1):128 (Fig. 1). Reprinted with permission.)
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Fig. 4.2 The percentage of femoral head lateral to Perkin line (A/B) quantitates the Reimer Migration Index.
3. 4. 5. 6. 7. 8. 9.
Oral medications for spasticity, dystonia Botulinum toxin for temporary tone management Selective dorsal rhizotomy for lower extremity spasticity Intrathecal pump for generalized spasticity Orthopedic muscle lengthening and transfer Osteotomies for bony deformity Arthrodesis of spine or feet if collapse impairs function
u Disorders of Spinal Cord Peripheral Nerves and Muscles Anterior Horn Cell Diseases (Table 4.1)
Neuropathies (Table 4.2)
Muscular Dystrophies (Table 4.3)
Table 4.1 Anterior Horn Cell Disorders Age at Diagnosis
Inheritance Pattern
Life Expectancy
Signs
Orthopedic Manifestations
Laboratories
Polio
Variable
None (infectious)
Related to level of involvement
Asymmetric flaccid paralysis, asymmetric or absent DTR
Contractures, scoliosis
Depends on phase of illness
Type I or Werdnig– Hoffman
Birth to 6 mo
Autosomal recessive gene defect on 5 q SMN gene
Most die in infancy
Marked general weakness, no head control, absent DTR normal sensation
Fractures
Type II
6–12 mo
Autosomal recessive gene defect on 5 q
Early to mid-adulthood
Normal until ~6 mo, independent head control in sitting position, never ambulate
Hip subluxation, scoliosis, contructure
EMG: high-amplitude polyphasic NCS: widespread fascicular pattern of denervation
Type III Kugelberg– Welander
1–2 yr
Autosomal recessive gene defect on 5 q
Over 45 yr
Normal until ~1 yr, ambulate until 2nd decade, then wheelchair, never can run or climb stairs
Kyphosis, scoliosis
DNA test for SMN, same as type II
Spinal muscular atrophy
Abbreviations: DTR, deep tendon reflex; EMG, electromyelography; NCS, nerve conduction study.
4 Neuromuscular Disorders in Pediatric Orthopedics 169
Disease
Disease
Age of Diagnosis
Inheritance Pattern
Guillain-Barré syndrome
Variable
Friedreich ataxia (spinocerebellar degeneration)
Before 10 yr
Life Expectancy
Signs
Orthopedic Manifestations
Laboratories
None; postviral ~5% mortality Ascending pain, parinfection esthesia, and weakness
Contractures
↑ CSF protein
90 degrees. Consider pin fixation. b. Type III: Percutaneous pin fixation (Fig. 5.7)—one medial and lateral or two lateral pins. Both pins should start distal to the fracture site.
Fig. 5.6 Documentation of the status of all nerves and circulation before treatment of supracondylar humerus fractures. This involves (A) checking active palmarflexion (median nerve); (B) flexion of distal interphalangeal joints of the index finger and thumb–anterior interosseous nerve; (C) dorsiflexion of the metacarpophalangeal joints–posterior interosseous nerve; (D) flexion of the fifth finger distal interphalangeal joint; or (E) crossing of index and second fingers– ulnar nerve.
5 Pediatric Trauma 189
Fig. 5.7 One technique of closed reduction and percutaneous pinning. Longitudinal traction is applied in slight flexion to correct angulation and yet allow visualization. Fluoroscopy receiver serves as platform.
Fig. 5.8 Desired pin placement for medial and lateral pin technique.
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The lateral pin should engage a portion of the capitellum, and the medial pin should be slightly medial and anterior on the epicondyle to avoid the ulnar nerve (Fig. 5.8). Make a small incision to clear a tract. If two lateral pins are used, one should cross the lateral third of the fracture, and one should cross the central third of the fracture (Fig. 5.9). c. If anatomic closed reduction is not possible, perform open reduction. Check alignment of the fracture using Baumann angle (Fig. 5.10A); normal is 72 ± 4 degrees. Also check the anterior humeral line (Fig. 5.10B), which should intersect the anterior one third to one half of the capitellum. d. Aftercare: May begin protected range of motion at around 3 weeks with temporary splint removal. Remove pins at 6 weeks. 4. Nerve injury a. Frequency: Radial > median > anterior interosseous > ulnar b. Treatment: If deficit is present before reduction, it is probably a neuropraxia resulting from the injury; proceed with closed reduction. If no return by 5 months after injury, then obtain electromyelogram; explore and perform neurolysis if no recovery.
Fig. 5.9 Lateral pin fixation for type III supracondylar humerus fractures.
5 Pediatric Trauma 191
B A
Fig. 5.10 (A) Baumann angle (normal, 72 degrees); (B) anterior humeral line should intersect anterior or middle third of capitellum.
5. Arterial insufficiency a. Reduce fracture; do not hyperflex. 1) If perfusion returns, pin fracture. 2) If perfusion does not return, perform an open exploration through an anterior Henry approach. 3) If artery is entrapped, release and watch. 4) If in spasm, use lidocaine. 5) If an intimal tear occurs, repair. 6) If transected, vein graft. b. Measure compartment pressures after reperfusion and perform fasciotomy if needed.
Bibliography u General Wilkins KE. Elbow fractures. In Wilkins KE, Beaty J.H., eds. Fractures in Children Philadelphia: Lippincott; 2006.
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u Lateral Condyle Fracture Badelon O, Bensahel H, Mazda K, Vie P. Lateral humeral condylar fractures in children: a report of 47 cases. J Pediatr Orthop. 1988;8(1):31–34 Flynn JC. Nonunion of slightly displaced fractures of the lateral humeral condyle in children: an update. J Pediatr Orthop. 1989;9(6):691–696 Song KS, Kang CH, Min BW, Bae KC, Cho CH. Internal oblique radiographs for diagnosis of nondisplaced or minimally displaced lateral condylar fractures of the humerus in children. J Bone Joint Surg Am. 2007;89(1):58–63
u Medial Epicondyle Fracture Farsetti P, Potenza V, Caterini R, Ippolito E, Caterini R, Ippolito E. Long-term results of treatment of fractures of the medial humeral epicondyle in children. J Bone Joint Surg Am. 2001;83-A(9):1299–1305 Josefsson PO, Danielsson LG. Epicondylar elbow fracture in children. 35-year follow-up of 56 unreduced cases. Acta Orthop Scand. 1986;57(4):313–315
u Elbow Dislocation Carlioz H, Abols Y. Posterior dislocation of the elbow in children. J Pediatr Orthop. 1984;4(1):8–12 Fowles JV, Kassab MT, Douik M. Untreated posterior dislocation of the elbow in children. J Bone Joint Surg Am. 1984;66(6):921–926 Nestor BJ, O’Driscoll SW, Morrey BF. Ligamentous reconstruction for posterolateral rotatory instability of the elbow. J Bone Joint Surg Am. 1992;74(8):1235–1241
u Radial Head/Neck Fractures Metaizeau JP, Lascombes P, Lemelle JL, Finlayson D, Prevot J. Reduction and fixation of displaced radial neck fractures by closed intramedullary pinning. J Pediatr Orthop. 1993;13(3):355–360 Steele JA, Graham HK. Angulated radial neck fractures in children: a prospective study of percutaneous reduction. J Bone Joint Surg Br. 1992;74(5):760–764
u Olecranon Fractures Dormans JP, Rang M. Fractures of the olecranon and radial neck in children. Orthop Clin North Am. 1990;21(2):257–268
u Supracondylar Fractures Culp RW, Osterman AL, Davidson RS, Skirven T, Bora FW Jr. Neural injuries associated with supracondylar fractures of the humerus in children. J Bone Joint Surg Am. 1990;72(8):1211–1215 Omid R, Choi PD, Skaggs DL. Supracondylar humeral fractures in children. J Bone Joint Surg Am. 2008;90(5):1121–1132 Pirone AM, Graham HK, Krajbich JI. Management of displaced extension-type supracondylar fractures of the humerus in children. J Bone Joint Surg Am. 1988;70(5): 641–650 Skaggs DL, Cluck MW, Mostofi A, Flynn JM, Kay RM. Lateral-entry pin fixation in the management of supracondylar fractures in children. J Bone Joint Surg Am. 2004; 86-A(4):702–707
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u Pediatric Hand and Wrist Fractures Hand Fractures 1. Distal phalanx fractures a. b. c. d. e.
Pediatric mallet finger: Often an open injury Clean thoroughly Replace nail under fold Closed reduction or ORIF of the fracture as indicated Follow-up to rule out infection
2. Phalangeal neck (subcondylar) fractures a. Principles 1) Most occur in proximal phalanx. 2) Volar angulation is the most common. 3) Minimal remodeling occurs in this region. b. Treatment of displaced fracture 1) Closed reduction 2) Transarticular percutaneous pinning for 3 weeks 3) Buddy-tape for 1 week. 4) Late presentation: May openly reconstruct up to 4 weeks post fracture c. Malunion: Loss of flexion may occur if malposition with bony impingement is allowed to persist. d. Treatment: Volar approach and removal of bony block to flexion 3. Phalangeal shaft fractures a. Less common in children than adults b. May accept 10 degrees dorsal/palmar angulation c. Immobilization: short arm cast/splint 4. Metacarpophalangeal joint injuries a. Collateral ligament does not protect physis of proximal phalanx or metacarpal head. b. “Extra octave” fracture of small finger 1) Reduce using pencil in web space as a fulcrum. 2) Reduction should be maintained when pressure is released. 3) Hold with rolled cotton gauze between digits.
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c. Intra-articular fractures 1) If fragment is greater than 25% of the joint surface or involves any tendon insertion, it needs to be reduced to within 2 mm of anatomic alignment. 2) Growth arrest is seen in less than 1% of patients. d. Dislocation 1) Often complex dorsal dislocation a) Volar plate entrapped b) Joint space wide, diaphyses parallel c) Sesamoids interposed d) Skin dimpled on volar surface 2) Treatment a) One or two attempts at closed reduction b) Open reduction: Volar or dorsal approach c) Incise superficial transverse ligament lateral to volar plate 5. Pediatric thumb metacarpal fractures a. Metaphyseal or Salter I and II fractures: Attempt closed reduction and cast; pin if unacceptable b. Pediatric Bennett fractures: Closed reduction or ORIF if more than 1 mm displacement c. Gamekeeper’s fracture 1) Stener lesion, usually a Salter III type 2) ORIF if displaced more than 1 mm or normal radiographs 3) With positive stress test at 45 degrees of flexion 6. Scaphoid fractures a. Much less frequent in children than adults b. May occur with distal radius fracture c. If diagnosis is made within 0 to 3 months, it will usually heal with a short-arm thumb spica cast. d. Delayed union of more than 3 months → bone graft
Distal Radial Physeal Fractures 1. Salter I and II are most common. 2. One or two gentle reductions + with adequate analgesia or anesthesia
5 Pediatric Trauma 195
3. Immobilize in neutral position or pronation. 4. Growth arrest is rare. 5. Complications a. Compartment syndrome b. Median nerve injury
Bibliography Crick JC, Franco RS, Conners JJ. Fractures about the interphalangeal joints in children. J Orthop Trauma. 1987;1(4):318–325 Simmons BP, Peters TT. Subcondylar fossa reconstruction for malunion of fractures of the proximal phalanx in children. J Hand Surg Am. 1987;12(6):1079–1082 Waters PM. Operative carpal and hand injuries in children. J Bone Joint Surg Am. 2007;89(9):2064–2074
u Spine Fractures Cervical Spine Fractures 1. General principles a. Child should be transported on a special backboard to accommodate large head: Recess under head or lift under shoulders b. Obtain radiographs if 1) Unconscious patient 2) Neck pain 3) Head or facial bruising in motor vehicle accident c. Recommended films 1) Lateral, anteroposterior, open mouth 2) Obliques only if dislocation or subluxation is suspected d. Normal values 1) See Chapter 1’s Fig. 1.13. Also refer to this section for normal ossification patterns e. Algorithms are shown for “clearing” (ruling out injury) in the trauma patient with normal radiographs but with tenderness or altered consciousness (Figs. 5.11, 5.12, 5.13, and 5.14).
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Awake, alert No neck pain No midline tenderness Normal neurologic examination Intoxication Distracting injuries
No
Yes
Canadian cervical spine functional test: 45° right and left rotation
Temporarily nonassessable
Pass
Fail
Cervical spine cleared Remove collar Discontinue restrictions
Evaluate as symptomatic patient
Fig. 5.11 Evaluation of the asymptomatic blunt trauma patient. (From Anderson PA, Gugala Z, Lindsey RW, Schoenfeld AJ, Harris MB. Clearing the cervical spine in the blunt trauma patient. J Am Acad Orthop Surg. 2010;18(3):149–159 (Fig. 1). Reprinted with permission.)
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Asymptomatic Intoxication Distracting injury Urgent clearance needed
No
Yes
Reassess with clinical examination 24 to 48 hours after treatment for distracting injuries or return of normal mentation
Evaluate as obtunded patient
Clinical examination
Negative
Positive
Canadian cervical spine functional test: 45° right and left rotation
Pass Cervical spine cleared Remove collar Discontinue restrictions
Evaluate as symptomatic patient
Fail Evaluate as symptomatic patient
Fig. 5.12 Evaluation of the temporarily unassessable trauma patient. (From Anderson PA, Gugala Z, Lindsey RW, Schoenfeld AJ, Harris MB. Clearing the cervical spine in the blunt trauma patient. J Am Acad Orthop Surg. 2010;18(3):149– 159 (Fig. 2). Reprinted with permission.)
198 Handbook of Pediatric Orthopedics
Neck pain Midline tenderness Neurologic signs or symptoms Radiologic imaging (required)
Option 1: Three radiographic views of cervical spine
Option 2: MDCT
Imaging test
Negative
Positive
Unexplained neurologic deficits Suspected ligamenious injury
MDCT if needed Spine consultation Collar immobilization Activity restrictions
No
Maintain collar Follow-up examination (2 weeks) Three radiographic views of cervical spine or flexion-extension radiographs
Negative
Cervical spine cleared Remove collar
Positive
Possible MRI or treat as cervical spine injury
Yes MRI (fat suppression or STIR)
Positive
Spine consultation Collar immobilization Activity restriction
Fig. 5.13 Evaluation of the symptomatic trauma patient. (From Anderson PA, Gugala Z, Lindsey RW, Schoenfeld AJ, Harris MB. Clearing the cervical spine in the blunt trauma patient. J Am Acad Orthop Surg. 2010;18(3):149–159 (Fig. 3). Reprinted with permission.)
5 Pediatric Trauma 199
Altered mental status Prolonged intubation Psychiatric disturbance Unable to cooperate Imaging (required)
MDCT reformations
Negative
Option 1: Clear cervical spine Discontinue collar and restrictions
Positive
Option 2: MRI
Spine consultation Collar immobilization Activity restrictions
MRI (fat suppression or STIR)
Negative
Clear cervical spine Discontinue collar and restrictions
Positive
Spine consultation Collar immobilization Activity restrictions
Fig. 5.14 Evaluation of the obtunded trauma patient. (From Anderson PA, Gugala Z, Lindsey RW, Schoenfeld AJ, Harris MB. Clearing the cervical spine in the blunt trauma patient. J Am Acad Orthop Surg. 2010;18(3):149–159 (Fig. 4). Reprinted with permission.)
200 Handbook of Pediatric Orthopedics
2. Atlanto-occipital displacement (Fig. 5.15) a. Usually skull is distracted and displaced forward. Suspect if dens– basion distance is greater than 12 mm or occipital condyles not resting in the superior facets of the atlas, or the Power ratio is greater than 1. Confirm with CT or magnetic resonance imaging. Document neurologic status. b. Immobilize with recessed backboard and minimal or no traction. c. Fusion of occiput to C1 or C2 is the most commonly accepted treatment. 3. Odontoid fracture a. Reduce and hold in halo or Minerva for 8 weeks; then a Philadelphia collar for 4 weeks 4. Atlas (C1–Jefferson) fracture a. Minimum (7 mm) is seen, then traction for 4 weeks followed by collar 5. C1-C2 rotatory subluxation (Fig. 5.16) a. CT scan is best study to confirm diagnosis b. Symptom duration less than 1 week → collar, analgesics, bed rest, exercises to reduce c. Symptom duration longer than 1 week → halter traction
Fig. 5.15 Atlanto-occipital displacement.
5 Pediatric Trauma 201
Fig. 5.16 C1-C2 rotatory subluxation.
d. Symptom duration longer than 1 month → halo traction, attempt reduction. Fuse in situ if not reducible 6. Transverse ligament insufficiency or os odontoideum (Fig. 5.17) a. Assess with flexion–extension views 1) 3 to 4 mm: Normal
Fig. 5.17 Transverse ligament insufficiency.
202 Handbook of Pediatric Orthopedics
2) 4 to 8 mm: Collar, restrict activities 3) More than 8 mm or any neurologic abnormalities → posterior fusion C1-C2 7. C2 pedicle fracture (Hangman’s) a. If C2-C3 disk is intact, immobilize in collar or halo. b. If C2-C3 disk disrupted, consider anterior spine fusion.
Thoracic and Lumbar Spine Fractures 1. Compression fracture a. If less than 20%, mobilize as tolerated. b. If greater than 20%, thoracolumbar spinal orthosis for comfort; mobilize as tolerated. 2. Burst fractures a. If neurologically normal, cast 6 to 8 weeks and then mobilize as tolerated. b. If neurologic deficit is present, decompress anteriorly or posteriorly and fuse. 3. Flexion–distraction (chance) (seatbelt) injuries (Fig. 5.18) a. Posterior elements distracted through facets, lamina, or pedicles. Minimal to no compression anteriorly b. Treatment: Attempt reduction in extension and immobilize for 6 to 8 weeks. c. If reduction is not obtained or is still unstable or if significant abdominal injury exists, then fuse.
u Femoral Shaft Fractures Background Principles 1. Mechanism: Pedestrian struck by car; fall or sports; passenger in motor vehicle accident 2. Acceptable reduction: Varus/valgus of up to 10 degrees, anterior/posterior bow of 20 degrees 3. Overgrowth of about 1 cm occurs between ages 2 and 10 years. 4. Family factors are important in choosing treatment. 5. Consider child abuse if patient is younger than 2 years. 6. Hip spica cast is not a good way to maintain length.
5 Pediatric Trauma 203
Fig. 5.18 Flexion-distraction (Chance) (seatbelt) injury.
Treatment 1. Age younger than 6 years: Resting overlap less than 2 cm or telescope test shows less than 3 cm of shortening: Yes → spica cast No → traction (in hospital or at home) or external fixator 2. Age 6 to 10 years a. If resting overlap is less than 2 cm or telescope test shows less than 3 cm of shortening, a spica cast is an option if the parents choose it. b. Preferred options: 1) Flexible IM rods/plate/fixator 2) Traction: in hospital or at home 3. Age older than 10 years a. IM rod with careful preparation of entry hole to avoid disrupting vessels at femoral neck (trochanteric entry) b. External fixator c. Plate
204 Handbook of Pediatric Orthopedics
Time to Union (Mean) 1. 2. 3. 4. 5.
Infant: Less than 4 weeks. Age 2 to 4 years: 4 to 6 weeks. Age 4 to 6 years: 6 weeks. Age 6 to 8 years: 6 to 8 weeks. Times are longer for open or high-energy injuries.
Subtrochanteric Fractures 1. 2. 3. 4. 5.
Overgrowth also occurs here: 1 cm on the average. One can accept angulation of 25 degrees in any plane. Fracture tends to develop anterior bow. Fracture is harder to image in cast. Treatment a. ORIF, plate b. External fixator c. Traction for 3 weeks in 90-degree flexion, then spica in 90–90 flexion or
u Physeal Injuries About the Knee Normal Anatomy and Growth 1. Length of lower limbs doubles between 4 years and maturity. 2. Distal femoral physis a. b. c. d.
Quadripodal shape Ligaments concentrate stress on this physis. Growth is 1 cm/year till age 13½ (girls), 15½ (boys). Blood supply to physis comes from epiphyseal vessels primarily, with some contribution from periosteal vessels.
3. Proximal tibial physis a. An anterior extension continues down to the tubercle. b. Ligaments, fibula, and semimembranosus insertion protect physis. c. Growth is 8 mm/year.
Distal Femoral Physeal Fracture 1. Most common physeal injury about the knee 2. Mechanism
5 Pediatric Trauma 205
a. Hyperextension, or b. Valgus 3. Findings a. Hemarthrosis, especially in Salter III, IV b. May be missed in polytrauma patient. c. Ligamentous injury may coexist. 4. Radiographs a. Obliques and tunnel views if needed b. Stress view if occult fracture suspected c. Plain tomograms or CT for complex Salter III and IV if fracture pattern or displacement is in question d. When to obtain arteriogram 1) If vascular examination is abnormal 2) Proximal tibia physeal fracture 3) Incidence of vascular injury ≤ 1% 4) In most cases, arteriogram is not necessary; especially in varus– valgus injuries. Instead, check circulation before and after reduction and instruct caregivers in monitoring it. 5. Treatment a. b. c. d. e. f.
Gentle closed reduction One may accept 5 degrees varus/valgus in Salter types I and II Open reduction if irreducible closed Pin if unstable ORIF all displaced type IV fractures Ensure physeal alignment by direct inspection as well as fluoro (at fracture and periphery). g. Immobilization 1) Long leg cast if limb is slender and fracture is stable 2) Spica cast otherwise h. Begin range of motion by 6 weeks 1) Follow-up at least 1 year to rule out growth plate injury 6. Results a. 25 to 50% have length discrepancy greater than 1 cm. b. 25% have angular deformity >5 degrees.
206 Handbook of Pediatric Orthopedics
7. Treatment of physeal bridge a. Imaging 1) Growth lines visible on plain film should be present and parallel to physis if growth is normal. 2) Tomograms (plain, not CT) if bridge is suspected 3) MRI: Discuss with radiologist before study b. Indications for resection: 1) Area of bar is less than 50% of physeal area. 2) Growth remaining more than 2 years
Proximal Tibial Physis Injury 1. One quarter as common as distal femur physeal injury; 5% have popliteal, peroneal injuries. 2. Mechanism a. Hyperextension b. Valgus c. 50% occur in sports. 3. Treatment a. Closed versus open reduction; based on standard criteria b. Close vascular monitoring
Tibial Tubercle Fractures 1. Background a. b. c. d.
Many have history of Osgood–Schlatter lesion. Frequency in males is greater than in females. Usual age range is 14 to 16 years. Almost always seen in jumping sports
2. Treatment a. Closed if minimally displaced and patient can activity actively extend knee b. ORIF 1) Clear bed of interposed tissue. 2) Use screw if fragment is large and the patient is near maturity. 3) Otherwise, suture tendon and periosteum.
5 Pediatric Trauma 207
3. Complications: Rarely seen a. Recurvatum: Only if patient is very young (i.e, younger than 11 years) b. Lack of flexion
Bibliography Burkhart SS, Peterson HA. Fractures of the proximal tibial epiphysis. J Bone Joint Surg Am. 1979;61(7):996–1002 Christie MJ, Dvonch VM. Tibial tuberosity avulsion fracture in adolescents. J Pediatr Orthop. 1981;1(4):391–394 Flynn JM, Schwend RM. Management of pediatric femoral shaft fractures. J Am Acad Orthop Surg. 2004;12(5):347–359 Hedequist D, Bishop J, Hresko T. Locking plate fixation for pediatric femur fractures. J Pediatr Orthop. 2008;28(1):6–9 Ogden JA, Tross RB, Murphy MJ. Fractures of the tibial tuberosity in adolescents. J Bone Joint Surg Am. 1980;62(2):205–215 Riseborough EJ, Barrett IR, Shapiro F. Growth disturbances following distal femoral physeal fracture-separation. J Bone Joint Surg Am. 1983;65:855–893
u Physeal Injuries About the Ankle Background 1. Normal growth a. Distal tibial and fibular epiphyses appear at around the age of 2 years and close at age 15 to 16 years. b. Anterolateral portion closes last. c. Distal fibular physis is normally at the level of tibial plafond.
Fracture Classification (Dias) 1. 2. 3. 4. 5. 6. 7.
SER (supination–external rotation) PER (pronation–external rotation) SPF (supination–plantar flexion) SI (supination–inversion) Axial loading Tillaux Triplane
Nonarticular Physeal Fractures 1. Closed versus open reduction: Standard criteria (5 degrees varus/valgus is acceptable)
208 Handbook of Pediatric Orthopedics
2. Check rotation radiographically and clinically by thigh–foot angle. 3. Use long leg cast in most cases if displaced.
Tillaux Fractures 1. Anterior inferior tibiofibular ligament avulses unfused epiphyseal fragment. 2. Mechanism = SER 3. Treatment: Reduce with internal rotation gap greater than 2 mm → ORIF
Triplane Fracture 1. Mechanism usually SER 2. May be 2, 3, or 4-part 3. Concern is mainly articular congruity rather than growth remaining (most patients with this fracture are nearing maturity). 4. Treatment a. b. c. d.
Attempt closed reduction. If it looks all right, get CT afterward to confirm. ORIF if more than 2-mm spread or any vertical displacement Anterolateral incision; Reduce posteromedial fragment first, then medial incision if needed.
Salter IV 1. Reduce and fix if there is any longitudinal displacement or more than 2-mm spread.
Physeal Arrest 1. Distal tibia is third most common site of growth arrest after fracture. a. Represents 25% of all physeal bars b. May occur with Salter II as well as III, IV, and V 2. Implications a. Counsel family of this risk at time of fracture b. Follow up for a year or longer after injury 3. Partial arrest a. Calculate potential angulation based on growth remaining b. Some guidelines
5 Pediatric Trauma 209
1) 8 mm = 10-degree deformity will occur if bar forms before age 13½ (boys) or 11½ (girls) 2) 1-cm growth remains after age 13 (boys), age 11 (girls) 3) Refer to growth remaining chart (Fig. 1.23). 4. Bar resection versus epiphysiodesis a. Latter simpler, more predictable b. Patient’s choice c. There is less need to do resection than in other areas of skeleton because length considerations are lessened.
Bibliography Cooperman DR, Spiegel PG, Laros GS. Tibial fractures involving the ankle in children: the so-called triplane epiphyseal fracture. J Bone Joint Surg Am. 1978;60(8):1040– 1046 Dias LS, Giegerich CR. Fractures of the distal tibial epiphysis in adolescence. J Bone Joint Surg Am. 1983;65(4):438–444 Kärrholm J, Hansson LI, Selvik G. Longitudinal growth rate of the distal tibia and fibula in children. Clin Orthop Relat Res. 1984;191(191):121–128 Khoshhal KI, Kiefer GN. Physeal bridge resection. J Am Acad Orthop Surg. 2005;13(1): 47–58 Kling TF Jr, Bright RW, Hensinger RN. Distal tibial physeal fractures in children that may require open reduction. J Bone Joint Surg Am. 1984;66(5):647–657 Schnetzler KA, Hoernschemeyer D. The pediatric triplane ankle fracture. J Am Acad Orthop Surg. 2007;15(12):738–747
210 Handbook of Pediatric Orthopedics
6 Normal Values and Medications This chapter includes laboratory values and medications the pediatric orthopedic surgeon needs to know. Pediatric dosages for medication must be calculated based on body weight. Normal laboratory values vary according to age. When giving agents that affect respiration, a plan should also be in place for monitoring and treatment of any adverse responses.
u Normal Laboratory Values (Tables 6.1 and 6.2)
u Antibiotics (Table 6.3)
u Other Medications Frequently Used in Pediatric
Orthopedics
(Table 6.4)
Table 6.1 Normal CBC by Age Age
HCT (%) mean – 2 SD
WBC/mm X 100 Mean ± 2 SD (Normal Ranges)
Newborn
51 (42)
18.1 (9–30)
6 mo
36 (31)
11.9 (6–17.5)
6–24 mo
36 (33)
10.6 (6–17)
2–6 yr
37 (34)
8.5 (5–15.5)
>6 yr
40 (35)
8.1 (4.5–13.5)
Abbreviations: HCT, hematocrit; SD, standard deviation; WBC, white blood cell count.
6 Normal Values and Medications 211
Table 6.2 Normal Lab Values by Age Parameter
Normal values
Sodium
135–145 mg/dL
Potassium
3.5–5.0 mg/dL
Phosphorus
8–10.5 mg/dL
Alkaline phosphatase Infant
150–400 U/L
2–10 yr
100–300 U/L
11–18 yr (male)
50–375 U/L
11–18 yr (female)
30–300 U/L
Erythrocyte sedimentation rate
1–5 mm/h
Creatinine Infant
0.2–0.4 mg/dL
Child
0.3–0.7 mg/dL
Adult
0.5–1.0 mg/dL
Glucose 1 wk–16 yr
60–105 mg/dL
>16 yr
70–115 mg/dL
Albumin 3–4 mo
2.8–5.0 mg/dL
1 yr
3.5–5.0 mg/dL
2 yr–adult
3.3–5.8 mg/dL
ALT (SGPT) 11 yr, 10 mg Calcium carbonate (Os-Cal, Tums) Chloral hydrate - sedative/hypnotic
50–100 mg/kg/dose (continued on p. 218)
6 Normal Values and Medications 217
Ascorbic acid (vitamin C)
Table 6.4 (continued from p. 217) Dose
Interval
Cimetidine
Children: 20–40 mg/kg/day
q8–12h
Route
Contraindicated in renal, cardiac disease
Adults: 1.2 g/day Codeine
1 mg/kg/dose
Dantrolene for malignant hyperthermia crisis
1 mg/kg; repeat until signs & symptoms normalize, up to 10 mg/kg
Diazepam (Valium)
Sedative: 0.1–0.3 mg/kg/day
q4h
p.o./i.m.
q2–4h
i.m./p.o.
Anticonvulsant: 0.1–0.3 mg/kg/dose
i.v. bolus
Rate should not exceed 5 mg/min Maximum dose: infants and toddlers, 5 mg. Older children, 15 mg. May repeat q15 min, x2 Diphenhydramine (Benadryl) Dimetapp (decongestant/antihistamine)
Ducosate sodium - laxative (Colace)
Comments/Side Effects
Child: 5 mg/kg/day
q6h
p.o., i.v.
Adult: 100–200 mg/day
q6h
p.o., i.v.
1 mo–2 yr: 1.25 mL
q6–8h
p.o.
2–4 yr: 3.75 mL
q6–8h
p.o.
4–12 yr: 5 mL
q6–8h
p.o.
>12 yr: 5–10 mL
q6–8h
p.o.
1 tab
q12h
p.o.
< 3 yr: 10–40 mg/24 h
q6–12h
p.o.
3–6 yr: 20–60 mg/24 h
q6–12h
p.o.
218 Handbook of Pediatric Orthopedics
Medication
Ducosate and casanthranol (laxative) (Pericolace)
6–12 yr: 40–120 mg/24 h
q6–12h
p.o.
>12 yr: 50–200 mg/24 h
q6–12h
p.o.
5–10 mL
q.h.s.
p.o.
Fentanyl
0.5–3.0 µg/kg/dose
q1h
i.v.
Ferrous sulfate
Drops (15 mg Fe/0.6 mL)
q8h
p.o.
q6–12h
i.v.
Give over 3 min
Syrup (18 mg Fe/5 mL) Elixir (44 mg Fe/5 mL) Tablet (60 mg Fe/tab) Dose 3 mg Fe/kg/24 h Dose 1 mg/d
Furosemide (Lasix)
Child: 1 mg/kg/dose (may increase by 1 mg/kg/dose)
p.o.
Adult: 20–80 mg/dose Haloperidol (sedative)
0.01–0.1 mg/kg
q2h
p.o.
Hydromorphone (Dilaudid)
1–4 mg
q4–6h
p.o., i.v., i.m.
Ibuprofen (Motrin, Advil)
20–40 mg/kg/day (suspension = 100 mg/tsp) (tablets = 200, 400, 600 mg)
q6–8h
p.o.
Ketamine (hypnotic)
4–8 mg/kg
i.m.
0.5–2 mg/kg
i.v.
Fewer side effects than morphine sulfate
May cause laryngospasm, respiratory depression (continued on p. 220)
6 Normal Values and Medications 219
Folic acid (vitamin)
Medication
Dose
Interval
Route
Comments/Side Effects
Ketorolac (Toradol)
Child: 1 mg/kg load, 0.5 mg/kg dose
q6h
i.v.
Do not use parenterally > 5 days
Adult: 10 mg
q6h
p.o.
Lidocaine - local anesthetic
Up to 1 mg/kg for regional block
Meperidine HCL (Demerol)
Child: 1–1.5 mg/kg/dose
q6h
p.o./i.m./i.v.
Adult: 50–150 mg/dose
q6h
p.o./i.m./i.v.
Methylprednisolone (steroid dose for spinal cord injury)
30 mg/kg bolus, then 5.4 mg/kg/h × 23 h
Midazolam (sedative/amnestic; Versed)
0.05–0.15 mg/kg/dose
Morphine sulfate
I.v.
q4h
i.m., s.c.
0.1–0.3 mg/kg/dose
q4h
i.m., s.c.
0.1 mg/kg/dose
q2h
i.v.
1.0 mg/kg/dose
Naloxone (Narcan)
p.r.
Continuous: 0.025–2 mg/kg/h
Short-acting, may need redose
0.01–0.1 mg/kg/dose Up to maximum 2 mg/dose. Repeat q 3–5 min Naproxen
10 mg/kg/day (suspension = 125 mg/tsp) (tablets - 250 & 375 mg)
q12h
p.o.
Nystatin (antifungal, topical)
Infants: 1 mL
q6h
p.o.
220 Handbook of Pediatric Orthopedics
Table 6.4 (continued from p. 219)
Children: 2–3 mL
q6h
p.o.
Ondansetron (Zofran)
.15 mg/kg/dose
q4h x3
i.v.
Oxycodone
Child: 0.05–0.15 mg/kg/dose
q6h
p.o.
Abuse potential, urinary retention
Adult: 5 mg 0.3 ml/kg/dose
Paragoric (analgesic)
0.25–0.5 mL/kg/dose
q6h
i.m./p.o./p.r. p.o.
Prochlorperazine (antiemetic; Compazine)
(>2 yr only) 0.4 mg/kg/day
q6–8h
p.o./p.r.
Promethazine (Phenergan)
Child: 0.25–0.5 mg/kg/dose
q4h
p.o./i.m./i.v.
Trimethobenzamide (antiemetic; Tigan)
20–45 kg 100 mg
q6–8h
p.o.
90 degrees) and abduction (for stability and safe zone >20 degrees). Use padding and molding over affected trochanter(s) to assist reduction. 8. Turn the edges of the cast down. Use tape to hold perineal edges of the liner and create a wide perineal window. Remove towels if used (Fig. 7.12D). 9. Window over abdomen or G-tube if needed 10. Radiographs or other imaging of hip or femur if indicated 11. Perform neurologic and vascular checks. 12. Arrange wheelchair and car seat.
u Ponseti Technique for Clubfoot Cast Indications The Ponseti technique is indicated for treatment of clubfoot in children under age 12 to 18 months who have not had surgery. It relies on guided correction of the three-dimensional malrotation of the foot and uses prolonged bracing to maintain the correction during growth. The goal is to avoid operative dissection of the bones of the foot and ankle, which could disturb growth and create incongruity. Invasive procedures are minimized and are directed at the tendons as indicated.
Procedures 1. The infant is kept as relaxed as possible, using judicious feeding and minimizing bright light, noise, or other stimuli. Manipulation assesses the components of cavus, adduction, hindfoot varus, and equinus. In bilateral cases, usually one foot is tighter than the other, and this should be noted. 2. Padding is applied, and the foot is held by one worker who is positioned on the lateral side of the foot being treated. The foot is held in the corrected position. Another worker applies the cast of plaster or soft fiberglass. The long-leg cast is applied from the tips of the toes to midthigh
242 Handbook of Pediatric Orthopedics
with the leg flexed 70 to 90 degrees. Avoid changing positions during this stage. 3. Final molding is performed before and during setting of the cast. This involves the following forces: a. Dorsiflexion of the first ray, with a finger under the metatarsal head and thumb over talar neck to correct the cavus (Fig. 7.13A) b. Abduction of the first ray to correct the adduction c. External rotation of the foot to correct the varus d. Gentle dorsiflexion of the ankle. e. Mold the above-knee portion of the cast to prevent slipping off. f. The cast is changed weekly until the malrotation and adduction are slightly overcorrected (Fig. 7.13B). If the hindfoot equinus does not correct beyond neutral into dorsiflexion, an Achilles’ tenotomy is indicated. This is needed in most cases, usually after three to five cast changes. Stress dorsiflexion lateral radiograph may be obtained if there is any question. g. Achilles’ tenotomy is performed under local anesthesia in the office (my preference) or in the operating room with sedation. The Achilles’ tendon is marked, 1 cm above the calcaneal insertion. It is infiltrated with local anesthetic. After 5 to 10 minutes, it is reprepped and incised with a #11 scalpel or Beaver blade. A distinct “give” should be felt. If not, redirect the blade slightly to ensure a complete tenotomy. Bleeding should be controlled with pressure over a sterile dressing. Then a new corrective cast is applied for 2 weeks. i. After the last cast, the feet are held in corrected position in a foot abduction orthosis. This is a bar with the feet held in 65 degrees of external rotation and 10 degrees of dorsiflexion on the involved side(s). It is worn full time for 2 months. After that, it is worn at night and nap time for 3 to 5 years, depending on the correction. Compliance with this phase is critical to success of the treatment. j. During the follow-up phase, a family member should work on stretching the foot into external rotation and eversion and dorsiflexion. As the child matures, instruct on active exercises in these directions. k. If recurrence is noted, repeat the casting protocol and resume bracing. l. If residual deformity is noted at around 4 to 5 years, this can usually be corrected by minimally invasive surgery. Equinus can be corrected by an open Achilles’ tendon lengthening. Internal rotation and adduction can be corrected by transfer of the anterior tibialis tendon to the third cuneiform or base of the third metatarsal. Any fixed deformity should be corrected first. m. Rarely, resistant cases may require open releases. n. Tips and tricks
7 Common Procedures 243
A Fig. 7.13 Ponseti method for correction of clubfoot.
B
C
244 Handbook of Pediatric Orthopedics
1. The fulcrum for the correction involves counter pressure over the talar head, not the calcaneocuboid joint. The goal is to rotate the foot around the talar head and then to dorsiflex it at the tibiotalar joint. 2. If the cast is repeatedly kicked off by the infant, use tincture of benzoin on the skin, increase the degree of knee flexion slightly, and increase the supracondylar molding. 3. If the subcutaneous padding of the foot is thin over the talar head, adhesive padding over this area should be used. 4. If the family is not able to ensure orthosis wear, meet with them more frequently. Use interpreters if needed. Involve other family members if available. 5. One reason for poor tolerance of abduction orthosis is residual deformity. Assess and consider further casting into an overcorrected position. 6. Several varieties of foot abduction orthoses are available. There are foot pieces of soft leather (available through www.mdorthopaedics .com). One design has hinges to allow some kicking movement. 7. If the infant will not tolerate the foot abduction orthosis, a singleleg knee-ankle-foot orthosis may be ordered that will hold the foot in external rotation and dorsiflexion.
u Minerva Cast Application Indications Maintenance of torticollis correction; immobilization of upper cervical spine injuries such as reduced or nondisplaced odontoid fracture
Equipment Adequate sedation or analgesia. Cast liner for head. Philadelphia collar to create padded contouring around chin (Fig. 7.14A). Padding for base of neck. Head position can be temporarily maintained using manual support (Fig. 7.14B) or halter (Fig. 7.14C). Hair may need to be trimmed. Thin cast material around the head and neck; wider for torso.
Procedure Maintain head position using halter or hands. Apply Philadelphia collar around neck. Add cast liner around torso. Apply cast and reinforce around neck. Trim and pad (Fig. 7.14D).
7 Common Procedures 245
A
B
Fig. 7.14 Minerva cast illustrations.
246 Handbook of Pediatric Orthopedics
C
D
Fig. 7.14 (continued)
Bibliography Hendrix RW, Lin PJ, Kane WJ. Simplified aspiration or injection technique for the sacroiliac joint. J Bone Joint Surg Am. 1982;64(8):1249–1252 Jameson SS, Kumar CS. The efficacy of combined popliteal and ankle blocks in forefoot surgery. J Bone Joint Surg Am. 2009;91(2):486–487, author reply 487 Jarrett GJ, Rongstad KM, Snyder M. Technique tip: popliteal nerve block by surgeon in the lateral decubitus position. Foot Ankle Int. 2004;25(1):37–38 Miskew DB, Block RA, Witt PF. Aspiration of infected sarco-iliac joints. J Bone Joint Surg Am. 1979;61(7):1071–1072 Siapkara A, Duncan R. Congenital talipes equinovarus: a review of current management. J Bone Joint Surg Br. 2007;89(8):995–1000
Index
Note: Page numbers followed by f and t indicate figures and tables, respectively. A Acetabular index, 62, 63f Acetaminophen, guidelines for pediatric use, 217t Achondroplasia, 134, 135f Acrocephalosyndactylies, 159 Acrodactyly, 104 Acromegaly, 104 Acrosyndactyly, congenital constriction bands and, 132 Adamantinoma, 124, 124f Advil. See Ibuprofen Aitken classification, of proximal femoral focal deficiency, 75–76, 75f Alanine aminotransferase, serum, normal values by age, 211t Albumin, serum, normal values by age, 211t Albuterol, guidelines for pediatric use, 217t Alkaline phosphatase, serum, normal values by age, 211t ALT. See Alanine aminotransferase Amikacin, guidelines for pediatric use, 212t Amniotic band syndrome. See Congenital constriction band syndrome Amoxicillin, guidelines for pediatric use, 212t Amoxicillin + clavulanic acid, guidelines for pediatric use, 212t Ampicillin, guidelines for pediatric use, 212t Amputations, in utero, 132, 133, 160 single, 160 Anatomy, neurologic, 3–8 Ancef. See Cefazolin Aneurysmal bone cyst, 124f, 125 and back pain, 116 pelvic, 125 scapular, 126 spinal, 125 Ankle flexion, during walking, 52, 53f fractures classification, 207 and physeal arrest, 208–209 physeal injuries about, 207–209 Ankylosing spondylitis (AS), and back pain, 116 Anterior horn cell disorders, 169t Antibiotic(s)
guidelines for pediatric use, 212t–216t for Lyme disease, 131 for musculoskeletal infection, 128t, 129 Apert syndrome, 159 and syndactyly, 158 Arachnodactyly congenital contractural, 145–146 in Marfan syndrome, 143–144 Archard syndrome, 146 Arnold classification, of musculoskeletal problems in hemophilia, 127–128 Arthritis enteropathic, and back pain, 116 septic, microbiology, 129, 129t Arthrocentesis, 131 Arthrogryposis, 147 clinical features, 147 and clubfoot, 94 and congenital scoliosis, 118 treatment, 147 Ascorbic acid, guidelines for pediatric use, 217t Aspartate aminotransferase, serum, normal values by age, 211t Aspirin, guidelines for pediatric use, 217t AST. See Aspartate aminotransferase Ataxia-telangiectasia (AT), 149–150 Atlantoaxial rotatory instability, 105–106 Atlanto-occipital displacement, 200, 200f Atlas (C1), development, 19, 19f Atlas-dens interval, 18, 18f Augmentin. See Amoxicillin + clavulanic acid Avascular necrosis in Cornelia de Lange syndrome, 140 of femoral head (See also Legg-CalvePerthes disease) differential diagnosis, 67–68 in Riley-Day familial dysautonomia, 140 with slipped capital femoral epiphysis, 73 AVN. See Avascular necrosis Axillary block, 230, 231f Axis (C2), development, 19–20, 20f B Back pain, in children, 115–117. See also Spondylolisthesis imaging for, 117 treatment, 117 warning signs of serious underlying disorder in, 117 Baclofen, guidelines for pediatric use, 217t
248 Index Baker cyst. See Popliteal (Baker) cyst Bardet-Biedl syndrome, 151 Baumann angle, 14, 15f, 190, 191f Beals syndrome, 145–146 Becker muscular dystrophy, 171t laboratory findings in, 164 Beckwith-Wiedemann syndrome, 151 Beclomethasone, guidelines for pediatric use, 217t Benadryl. See Diphenhydramine Bennett fracture, pediatric, 194 Bier block, 229 Birth fractures, of shoulder, 179 Bisacodyl, guidelines for pediatric use, 217t Bladder, neurogenic, 173 Bladder exstrophy, 77–78 classic, 77–78, 78f Blount disease, 122 and lower-limb length inequality, 55 Blue-rubber bleb nevus syndrome, 149 Bone cyst aneurysmal (See Aneurysmal bone cyst) unicameral (See Unicameral bone cyst) Brachial plexus, formation, 6, 7f Bruck syndrome, 157 C Cadence (gait) by age, 52 definition, 51 Café-au-lait spots fibrous dysplasia and, 142 in neurofibromatosis type 1, 153 Calcaneonavicular bar, 96, 97f Calcaneovalgus foot, 98–99 Calcium carbonate, guidelines for pediatric use, 217t Camurati-Engelmann disease, 141 Carbenicillin, guidelines for pediatric use, 212t Carpenter syndrome, 159 Catterall staging, of Legg-Calve-Perthes disease, 65–66, 66f Cauliflower ear, 136 CBC. See Complete blood count Ceclor. See Cefaclor Cefaclor, guidelines for pediatric use, 212t Cefadroxil, guidelines for pediatric use, 212t Cefamandole, guidelines for pediatric use, 212t Cefaperazone, guidelines for pediatric use, 213t Cefazolin, guidelines for pediatric use, 213t Cefobid. See Cefaperazone Cefotaxime, guidelines for pediatric use, 213t Cefoxitin, guidelines for pediatric use, 213t Ceftazidime, guidelines for pediatric use, 213t Ceftizoxime, guidelines for pediatric use, 213t Ceftriaxone, guidelines for pediatric use, 213t Cefuroxime, guidelines for pediatric use, 213t Center angle of Wilberg, 62–64, 63f Central core myopathy, 171t Centronuclear myopathy, 171t Cephalexin, guidelines for pediatric use, 213t Cephalothin, guidelines for pediatric use, 214t Ceptaz. See Ceftazidime Cerebral palsy, 166–168 anatomic types, 166 asymmetric diplegic, 166 causes, 166
definition, 166 diplegic, 166 extrapyramidal (dystonic or athetoid), 166 gross motor functional classification system for, 166, 167f hemiplegic, 166 hip subluxation rating in, 166, 168f and lower-limb length inequality, 55 mixed type, 166 monoplegic, 166 physiologic types, 166 pyramidal (spastic), 166 totally involved (quadriplegic), 166 treatment, 166–168 Cervical spine alignment, normal values for children, 18–19, 18f atlas (C1-Jefferson) fracture, 200 C1 (atlas), development, 19, 19f C2 (axis), development, 19–20, 20f C1-C2 rotatory subluxation, 200–201, 201f clearing, 195, 196f–199f C2 pedicle fracture (hangman’s), 202 development, 19–20, 19f, 20f fractures, 195–202 pseudosubluxation, 18, 18f transverse ligament insufficiency, 201–202, 201f vertebral bodies, anterior wedging, 18f, 19 Chance injury, spinal, 202, 203f Charcot-Marie-Tooth disease hypertrophic, 170t neuronal form, 170t Chiari malformation, 173 Child abuse, femoral shaft fracture in, 202 Chloral hydrate, guidelines for pediatric use, 217t Chloramphenicol, guidelines for pediatric use, 214t Chondroblastoma epiphyseal tumor of adolescence, 123, 124f Chondrodysplasia punctata, 137 Chondrolysis, with slipped capital femoral epiphysis, 73 Chondromyxoid fibroma, 123, 124f Chondrosarcoma, 124f, 125 of ribs, 125 Chordoma, vertebral body, 125 Chromosome abnormalities, 162–163 Cimetidine, guidelines for pediatric use, 218t Cipro. See Ciprofloxacin Ciprofloxacin, guidelines for pediatric use, 214t Claforan. See Cefotaxime Clavicle, medial, physeal closure, age at, 12 Cleidocranial dysplasia, 139 Clindamycin, guidelines for pediatric use, 214t Cloacal exstrophy, 77 Cloxacillin, guidelines for pediatric use, 214t Clubfoot, 94–95, 132–133, 147 cast, Ponseti technique for, 241–244, 243f contractural syndromes and, 147–148 DiMeglio scoring system for, 94, 94t Clubhand radial, 159 ulnar, 160 Coast of Maine appearance, fibrous dysplasia and, 142 Cobb angle, 108–109
Index 249 Codeine, guidelines for pediatric use, 218t Colace. See Docusate sodium Compazine. See Prochlorperazine Complete blood count, normal, by age, 210t Computed tomography (CT) of head, in trauma patient, indications for, 175 measurement of femoral anteversion, 37–39, 39f Congenital constriction band syndrome, 132–133, 159 amputations in, 160 clinical features, 132 and clubfoot, 94, 132–133 differential diagnosis, 132 incidence, 132 treatment, 133 Congenital contractural arachnodactyly, 145–146 Congenital vertical talus, 99–100, 100f, 147 Connective-tissue disorders (CTD). See also Ehlers-Danlos syndromes and clubfoot, 94 Conradi-Hunerman syndrome, 137 Contracture(s). See Arthrogryposis; Congenital contractural arachnodactyly Cornelia de Lange syndrome, 160 Brachmann de Lange type, 140 Coxa vara, 136, 139 developmental, 74–75 Creatine phosphokinase, serum, in neuromuscular disorders, 164 Creatinine, serum, normal values by age, 211t Cri-du-chat syndrome, 163 CRITOE mnemonic, 13, 14f Crouzon syndrome, 159 Cubitus valgus, in Turner syndrome, 162 Cyst(s). See also Aneurysmal bone cyst; Unicameral bone cyst popliteal (Baker), 89–91, 90f D Dactylitis, in sickle cell anemia, 150 Dantrolene, for malignant hyperthermia, guidelines for pediatric use, 218t DDH. See Developmental dysplasia of the hip (DDH) Deep venous thrombosis, prophylaxis, 178 Dejerine-Sottas disease, 170t Demerol. See Meperidine HCL Denver II Developmental Screening Test, 1–2 Dermatomes, 3, 4f Developmental coxa vara, 74–75 Developmental dysplasia of the hip (DDH), 55, 57–65, 151 Barlow test for, 57–58f evaluation of older child for, 59, 60f Galeazzi sign, 59, 60f and Klisic line, 59, 60f management, 64, 64f and Nélaton line, 59, 60f Ortolani test for, 57, 58, 58f physical examination for, in newborn, 57–59, 58f radiographic evaluation, 62–64, 63f risk factors for, 57 ultrasound for, 59–62, 61f
Diastrophic dwarfism, and clubfoot, 94 Diastrophic dysplasia, 136 Diazepam, guidelines for pediatric use, 218t Dicloxacillin, guidelines for pediatric use, 214t Dilaudid. See Hydromorphone DiMeglio scoring system, for clubfoot, 94, 94t Dimetapp, guidelines for pediatric use, 218t Diphenhydramine, guidelines for pediatric use, 218t Discitis, and back pain, 116 Distal metatarsal articular angle (DMAA), 101 Docusate and casanthranol, guidelines for pediatric use, 219t Docusate sodium, guidelines for pediatric use, 218t–219t Dolichostenomelia, in Marfan syndrome, 143 Double support, definition, 51 Down syndrome, 162 Doxycycline, guidelines for pediatric use, 214t Duchenne muscular dystrophy, 171t electrocardiography in, 165 laboratory findings in, 164 Dulcolax. See Bisacodyl Duricef. See Cefadroxil Dwarfism, rhizomelic, 134–136 Dyschondrosteosis, 139 Dysplasia epiphysialis hemimelica, 138 Dystrophia myotonia, 172t Dystrophia myotonica, electrocardiography in, 165 Dystrophin immunoblot, 164 E Ectopia lentis, in Marfan syndrome, 144 Ehlers-Danlos syndromes, 154, 155t Elbow(s) annular ligament entrapment, 184, 185f determination of skeletal age using, 14, 15f dislocations, 184, 192 flexion contracture, in Marfan syndrome, 144 fractures lateral condyle, 181–183, 182f, 192 medial epicondyle, 183–184, 192 olecranon, 192 radial head and neck, 185–186, 192 injuries, 181–192 Monteggia fracture-dislocation, 186–187, 187f nursemaid’s, 184, 185f ossification about, sequence of, 13, 14f Electrocardiography in Lyme disease, 131 in neuromuscular disorders, 165 Electromyography (EMG), in neuromuscular disorders, 164 Emery-Dreifuss dystrophy, 171t EMG. See Electromyography (EMG) Enchondroma, 124, 124f Enchondromas, multiple, 139 Enzyme-linked immunosorbent assay (ELISA), for Lyme disease, 131 Eosinophilic granuloma, 123, 124f and back pain, 116 pelvic, 125 Epidermoid, and back pain, 116 Epispadias, 77
250 Index Equinovarus, 136 Erythema chronic migrans, 131 laboratory findings in, 131 Erythrocyte sedimentation rate in Lyme disease, 131 malignancy and, 123 normal values, 211t Erythromycin, guidelines for pediatric use, 214t Escobar syndrome. See Multiple pterygium syndrome Ethambutol, guidelines for pediatric use, 215t Eulenberg disease, 172t Ewing sarcoma, 123, 124f, 125 and back pain, 116 pelvic, 125 of ribs, 125 scapular, 126 vertebral body, 125 F Factor VIII deficiency, 126–128, 150 dosage and administration, 126–127, 128t pharmacokinetics, 128t Factor IX deficiency, 126–128 dosage and administration, 126–127, 128t pharmacokinetics, 128t Fanconi anemia, 159 Fascioscapulohumeral dystrophy, 171t Femoral anteversion, 37–39, 37f, 38f measurement clinical, 37–39, 38f using biplane radiographs (Ogate method), 39–41, 40f, 41f, 42f using computed tomography, 37–39, 39f Femoral head, immature, idiopathic avascular necrosis. See Legg-Calve-Perthes disease Femoral joint angle, 43 Femoral nerve block, 232, 233f Femur. See also Slipped capital femoral epiphysis congenital short, 55 distal epiphysis, ossification, 9 growth, 25, 25f growth remaining, by skeletal age, 26f physeal fracture, 204–206 physis, normal anatomy and growth, 204 length, normal for boys, 27f for girls, 28f neck, varus deformity, 74–75 proximal epiphysis, ossification, 12 focal deficiency, 55, 75–77 growth, 25, 25f shaft fractures, 202–204 subtrochanteric fractures, 204 traction pin placement in, 225, 226f true neck-shaft angle, 41, 42f Fentanyl, guidelines for pediatric use, 219t Ferrous sulfate, guidelines for pediatric use, 219t Fetal alcohol syndrome, 161
Fetal hydantoin syndrome, 161 Fibrodysplasia ossificans progressiva, 141 Fibroma, chondromyxoid, 123 Fibrosarcoma, 124f, 125 Fibrous dysplasia, 142–143 clinical manifestations, 142–143 and endocrinopathies, 143 genetics, 142 imaging findings in, 143 and lower-limb length inequality, 55 monostotic, 142 pathophysiology, 142 pelvic, 125 polyostotic, 142 of ribs, 125 treatment, 143 Fibula distal normal growth, 207 physeal fractures, growth plate damage in, 30 growth, 25f hemimelia, 55 First rocker, definition, 51 Flagyl. See Metronidazole Flatfoot, 97–98 Folic acid, guidelines for pediatric use, 219t Foot. See also Flatfoot calcaneovalgus, 98–99 deformity, 136 (See also Clubfoot) with bladder exstrophy, 77 contractural syndromes and, 147–148 in Cornelia de Lange syndrome, 140 and developmental dysplasia of the hip, 59 and lower-limb length inequality, 55 macrodactyly, 103–105, 104f malformations, 157–160 pediatric, normal radiographic measurements, 50–51, 50f physeal closure in, age at, 13f secondary ossification centers, appearance of, 12f, 13f Foot progression angle, 48 measurement, 45f Fortaz. See Ceftazidime Forward-bend test, for spinal deformity, 108, 108f Fracture(s). See also Elbow(s), fractures ankle classification, 207 nonarticular physeal, 207–208 Salter IV, 208 atlas (C1-Jefferson), 200 and back pain, 116 birth, 179 bleeding after, in hemophiliac, control, 127 burst, 202 compression, 202 C2 pedicle (hangman’s), 202 femoral shaft, 202–204 subtrochanteric, 204 time to union (mean), 204 treatment, 203 hand, 193–194 lumbar spine, 202 odontoid, 200 physeal, classification, 174, 175f, 176f
Index 251 proximal humeral, 179–181 in Riley-Day familial dysautonomia, 140 scaphoid, 194 spinal, 195–202 (See also Cervical spine, fractures) thoracic spine, 202 Tillaux, 208 triplane, 208 Freeman-Sheldon syndrome, 148 and clubfoot, 94 Freiberg syndrome, 122 Friedreich ataxia, 170t electrocardiography in, 165 Furosemide, guidelines for pediatric use, 219t G Gait
cadence by age, 52 definition, 51 joint motion during (normal), 52, 53f mature, age at development, 52 muscle activity during, 53f, 54 normal, in children, 51–54 normal parameters, 52 normal walking, 51 velocity, by age, 52 Gait cycle, phases, 51, 51f, 52f Galeazzi sign, and developmental dysplasia of the hip, 59, 60f Gamekeeper’s fracture, 194 Gardner-Wells tongs, 229 Gentamicin, guidelines for pediatric use, 215t Genu valgus, in Turner syndrome, 162 Giant cell tumor, 124f, 125 Glasgow Coma Scale, 177, 178t Glioma(s), and back pain, 116 Glucose, serum, normal values by age, 211t Goldenhar syndrome, 152, 159 and congenital scoliosis, 117 Greater trochanter, ossification, 12 Growth achondroplasia and, 134, 135f age at cessation for boys, 29 for girls, 29 normal, 135f prediction, multiplier method for, 36t, 37 Growth norms for boys, 34, 34f for girls, 34, 35f Growth remaining, calculation, 21–25, 23f–24f, 26f for upper extremity, 33, 33f Guillain-Barré syndrome, 170t nerve biopsy in, 165 H Hallux valgus, 101–102 adolescent, 101 in Down syndrome, 162 juvenile, 101 Halo, pediatric, 229 Haloperidol, guidelines for pediatric use, 219t Halo-vest, pediatric, 229 Hand(s)
anomalies, contractural syndromes and, 147–148 fractures, 193–194 distal phalanx, 193 phalangeal neck (subcondylar), 193 phalangeal shaft, 193 malformations, 157–160 physeal closure in, age at, 12f secondary ossification centers, appearance of, 12f HCT. See Hematocrit Height achondroplasia and, 134, 135f adult, estimation, 29 multiplier method for, 36t, 37 normal growth patterns for, 135f Hemangioma, vertebral body, 125 Hemarthropathy acute (hemophiliac), treatment, 126 subacute (hemophiliac), treatment, 127 Hematocrit, normal, by age, 210t Hemiatrophy, and lower-limb length inequality, 55 Hemiepiphysiodesis, for angular deformities of knee, 84, 85f–86f Hemihypertrophy, 104 idiopathic, 151–152 and lower-limb length inequality, 55 Hemimelia fibular, 55 tibial, 55 and clubfoot, 94 Hemivertebra, 117 Hemoglobin C, 150 Hemoglobin S, 150 Hemoglobin SC, 150 Hemoglobin SS, 150 Hemophilia A, 126–128, 150 B, 126–128 musculoskeletal problems in, 126–128 Arnold classification, 127–128 radiographic evaluation, 127–128 Hereditary arthro-ophthalmopathy, 146 Hereditary motor and sensory neuropathy(ies), 170t Herring lateral pillar classification, of LeggCalve-Perthes disease, 66, 66f Hilgenreiner-epiphyseal angle, 75, 75f Hilgenreiner line, 62, 62f Hindfoot valgus, in Marfan syndrome, 144 Hip(s). See also Slipped capital femoral epiphysis anomalies, contractural syndromes and, 147–148 dislocation, 136 with bladder exstrophy, 77 in Down syndrome, 162 Tonnis grades, 62, 62f dysplasia (See also Developmental dysplasia of the hip (DDH)) in Riley-Day familial dysautonomia, 140 flexion, during walking, 52, 53f rotation, 48, 48f, 49f measurement, 48, 48f subluxation, in cerebral palsy, 166, 168f transient synovitis, 69–71
252 Index Hip arthrogram/aspiration, technique for, 234–236, 234f, 235f Hip spica cast application, 238–241, 239f–240f equipment for, 238 indications for, 238 preparation for, 238 Histiocytosis, vertebral body involvement in, 125 Hitchhiker thumb, 136 Holt-Oram syndrome, 159 Homocystinuria, 145 Humerus distal, articular surface, angle of, 14, 15f growth, 25f proximal fractures, 179–181 ossification, 179 physiology, 178 unicameral bone cyst, fracture through, 180–181 supracondylar fracture, 187–191, 192 arterial insufficiency with, 191 classification, 187 nerve injury with, 190 status of nerves and circulation with, pretreatment documentation, 188, 188f treatment, 188–190, 189f, 190f, 191f and ulna, normal alignment, 182f Hunter syndrome, 158t Hurler syndrome, 158t Hydromorphone, guidelines for pediatric use, 219t I Ibuprofen, guidelines for pediatric use, 219t Infection(s) and back pain, 116 musculoskeletal, 128–130 differential diagnosis, 128–129 empiric antibiotic therapy for, 128t, 129 evaluation, 128 microbiology, 129, 129t work-up for, 128 Inflammation, and back pain, 116 Insall ratio, 87, 87f Intracranial pressure, measurement, indications for, 177 K Kasabach-Merritt syndrome, 149 Keflen. See Cephalothin Keflex. See Cephalexin Kefzol. See Cefazolin Ketamine, guidelines for pediatric use, 219t Ketorolac, guidelines for pediatric use, 220t Kinematics, definition, 51 Kinetics, definition, 51 King classification, of scoliotic curve types, 112, 112f Klein’s line, 73 Klinefelter syndrome, 163 Klippel-Feil syndrome, 152–153, 159 and congenital scoliosis, 118 Klippel-Trenaunay-Weber syndrome, 148–149 and macrodactyly, 103
Klisic line, and developmental dysplasia of the hip, 59, 60f Knee angular deformities, 84, 85f–86f anomalies contractural syndromes and, 147–148 in nail-patella syndrome, 141 deformity, 136 discoid lateral meniscus, 88–89 complete type, 88 imaging, 88 incomplete type, 88 prognosis for, 89 signs and symptoms, 88 treatment, 89 Wrisberg ligament type, 88 flexion, during walking, 52, 53f physeal injuries about, 204–207 “snapping,” 88 sulcus angle, 86f, 87 Kohler disease, 122 Kugelberg-Welander disease, 169t Kyphosis contractural syndromes and, 147–148 in Marfan syndrome, 144 in Riley-Day familial dysautonomia, 140 L Laboratory values, normal, by age, 210t, 211t Langenskjold stages, of tibia vara, 79–80, 79f Larsen syndrome, 147 and clubfoot, 94 Lasix. See Furosemide Latex allergy, prevention, 223 items containing, 222t items free of, 222t Legg-Calve-Perthes disease, 65–69, 122 age distribution, 65 bilateral involvement in, 65 differential diagnosis, 67–68 epiphyseal extrusion in, 67, 67f Gage sign, 67 “head at risk” signs of Catterall, 67 Herring lateral pillar classification, 66, 66f and lower-limb length inequality, 55 Mose sphericity, 67 prognostic signs, 67 radiographic findings in, 65–67 signs and symptoms, 65 staging Catterall, 65–66, 66f Herring lateral pillar classification for, 66, 66f Stulberg rating, 67, 68f treatment, 68–69 Lenke classification, of scoliotic curve types, 112, 113f Leri-Weill disorder, 139 Leukemia(s) and back pain, 116 pelvic involvement in, 126 secondary skeletal involvement in, 123, 124f Lidocaine, guidelines for pediatric use, 220t Limb-girdle dystrophy, 171t Limb-length inequality. See also Lower-limb length inequality
Index 253 in Beckwith-Wiedemann syndrome, 151 multiple hereditary exostoses and, 138 in Ollier disease, 139 prediction, straight-line graphs for, 30, 31f, 32f Lipomeningocele, 77, 173 Lobster-claw hand, in Cornelia de Lange syndrome, 140 Loder classification, of slipped capital femoral epiphysis, 72 Loeys-Dietz syndrome, 147 and clubfoot, 94, 147 Long bones growth, 22, 25f growth curves for, 27f, 28f, 29 growth remaining curves, 29f, 30 physeal closure, age at, 9, 10f–11f secondary ossification centers, appearance of, 9, 10f–11f tumors, 124f, 125 Louis-Bar syndrome. See Ataxia-telangiectasia (AT) Lower extremity. See also Lower limb alignment, 43, 44f anatomic axis, 43 contributions to growth of long bones, 22, 25f mechanical axis, 43 motor innervation, 7–8, 9f normal anatomy and growth, 204 osteochondrosis, 122 rotation, clinical evaluation, 44f–49f, 46–48 Lower limb. See also Lower extremity lengths, multiplier method for, 36t, 37 Lower-limb length inequality, 55–57 acquired causes, 55 computed radiograph, 56 congenital causes, 55 measurement, 56, 56f block method, 56, 56f tape method, 56 scanogram, 56 treatment, 56–57 Lumbar spine, fractures, 202 Lyme disease, 130–132 acute stage, 130 chronic stage, 130 differential diagnosis, 131 endemic areas, 130 HLA association in, 130 imaging in, 131 physical findings in, 131 prognosis for, 132 signs and symptoms, 130 treatment, 131 M Macrodactyly, 103–105 in Proteus syndrome, 153, 153t Madelung deformity, 122 in Leri-Weill disorder, 139 Maffucci syndrome, 149 Malignancy, musculoskeletal, 123–126 Marfan syndrome, 143–145 cardiac involvement in, 144–145 CNS involvement in, 145 diagnostic criteria for, 143–144
genetics, 143 ocular involvement in, 144 pulmonary involvement in, 145 skeletal involvement in, 143–144 Maroteaux-Lamy syndrome, 158t MASS phenotype, 146 McCune-Albright syndrome, 143 Median nerve, testing, 8f Mefoxin. See Cefoxatin Melorheostosis, 141 Meperidine HCL, guidelines for pediatric use, 220t Merchant view, of knee, 85, 86f Metacarpophalangeal joint, injuries, 193–194 Metaphyseal-diaphyseal angle, in tibia vara, 80, 80f Metastatic disease, of ribs, 125 Metatarsophalangeal (MTP) angle, 101 Metatarsus adductus, 46–48 appearance, 44f in Down syndrome, 162 quantification, 45f Metatarsus primus varus, 101 Metatropic dysplasia, 137 Methicillin, guidelines for pediatric use, 215t Methylprednisolone, guidelines for pediatric use, 220t Metronidazole, guidelines for pediatric use, 215t Meyer dysplasia, 68 MHE. See Multiple hereditary exostoses Michelin tire baby syndrome, 132 Micromelia, in Cornelia de Lange syndrome, 140 Midazolam, guidelines for pediatric use, 220t Minerva cast application, 244, 245f–246f equipment for, 244, 245f indications for, 244 Mobius syndrome, 148 and clubfoot, 94 Monosomy X. See Turner syndrome Monteggia fracture-dislocation, of radial head, 186–187, 187f Morphine sulfate, guidelines for pediatric use, 220t Morquio-A syndrome, 158t Morquio-B syndrome, 158t Moseley straight-line graph for prediction of limb-length inequality, 30, 31f, 32f Motor development in infants and children ages 1 to 5, 2–3 milestones, norms for, 1, 1t referral criteria, 3 Motrin. See Ibuprofen MPV. See Metatarsus primus varus MTP. See Metatarsophalangeal (MTP) Mucopolysaccharidosis (MPS), 157, 158t Multiple enchondromas, 139 Multiple epiphyseal dysplasia, 137 Multiple hereditary exostoses, 138 Multiple pterygium syndrome, 148 and congenital scoliosis, 118 Multiplier method, for growth prediction, 36t, 37 Muscle biopsy, in neuromuscular disorders, 165 Muscular dystrophy(ies), 171t. See also Becker muscular dystrophy; Duchenne muscular dystrophy
254 Index Musculoligamentous strain, and back pain, 116 Myelomeningocele, 77, 173 Myopathy(ies) central core, 171t centronuclear, 171t congenital, 171t electromyography in, 164 laboratory findings in, 164 muscle biopsy in, 165 rod-body, 171t Myositis, laboratory findings in, 164 Myotonia congenita, 172t Myotonic disorders, 172t Myotonic dystrophy, 172t N Nager syndrome, 159 Nail-patella syndrome, 141 Nails, hypoplastic, 132 Naloxone, guidelines for pediatric use, 220t Naproxen, guidelines for pediatric use, 220t Narcan. See Naloxone Neer and Horowitz classification, of proximal humeral displacement, 179–180 Nélaton line, and developmental dysplasia of the hip, 59, 60f Nephroblastoma, hemihypertrophy and, 152 Nerve biopsy, in neuromuscular disorders, 165 Nerve block(s), 229–234 Nerve conduction studies, in neuromuscular disorders, 165 Neural tube defects, lumbosacral, 77 Neuroblastoma and back pain, 116 secondary skeletal involvement in, 123 Neurodevelopment norms for, 1–3 referral criteria, 3 Neurofibromatosis (NF), type 1, 153–154 congenital tibial dysplasia in, 91–92, 92f diagnostic criteria for, 153–154 and macrodactyly, 103 orthopedic considerations in, 154 spinal curve in, 154 Neurogenic bladder, 173 Neurologic anatomy, 3–8 Neuromuscular disorders, evaluation, 164–165 Neuropathy(ies), 170t electromyography in, 164 hypertrophic, nerve biopsy in, 165 muscle biopsy in, 165 NF-1. See Neurofibromatosis (NF), type 1 Nonossifying fibroma, 124, 124f Noonan syndrome, 162–163 Nucleus pulposus, herniated, and back pain, 116 Nursemaid’s elbow, 184, 185f Nystatin, guidelines for pediatric use, 220t–221t O Oculoauriculovertebral dysplasia. See Goldenhar syndrome Odontoid, physis, age at fusion, 18, 18f Odontoid fracture, 200 Odontoid hypoplasia, 136
OI. See Osteogenesis imperfecta Oligohydramnios, and clubfoot, 94 Ollier disease, 139 and lower-limb length inequality, 55 Omphalocele, 77 Ondansetron, guidelines for pediatric use, 221t Os-Cal. See Calcium carbonate Osgood-Schlatter disease, 122, 206 Os odontoideum, 136, 201–202, 201f Ossification center(s), secondary, appearance of, 9–17, 10f–11f Osteoblastoma and back pain, 116 pelvic, 125 scapular, 126 spinal, 125 Osteocartilaginous exostosis, 123, 124f Osteochondroma, 123 multiple hereditary exostoses and, 138 Osteochondromatosis, and lower-limb length inequality, 55 Osteochondroses, 122 Osteogenesis imperfecta, 155–157 and lower-limb length inequality, 55 Sillence types, 156 type I, 156 type II (lethal perinatal), 156 type III, 156 type IV, 156 type V, 156 type VI, 157 type VII, 157 type VIII, 157 Osteogenic sarcoma, and back pain, 116 Osteoid osteoma, 123, 124f and back pain, 116 spinal, 125 Osteomyelitis and back pain, 116 microbiology, 129, 129t and physeal arrest, and lower-limb length inequality, 55 Osteopathia striata, 142 Osteopenia, in Riley-Day familial dysautonomia, 140 Osteopoikilosis, 142 Osteosarcoma, 123, 124f, 125 pelvic, 126 vertebral body, 125 Overgrowth syndromes, 150–153 asymmetric, 151–153 generalized, 150–151 Oxacillin, guidelines for pediatric use, 215t Oxycodone, guidelines for pediatric use, 221t P Panner deformity, 122 Paraldehyde, guidelines for pediatric use, 221t Paramyotonia congenita, 172t Paregoric, guidelines for pediatric use, 221t Parenteral nutrition, for polytrauma patient, 177 Patella. See also Nail-patella syndrome double layer, 137 Patella alta, 87f Patellar tilt, 87 Patellofemoral disorders, 85–88
Index 255 differential diagnosis, 85 physical examination for, 85 radiographic evaluation, 85–87, 86f, 87f treatment, 87–88 Pectus carinatum, in Marfan syndrome, 143 Pectus excavatum, in Marfan syndrome, 143 Pelvic deformity, with bladder exstrophy, 77–78, 78f Pelvis, tumors, 125–126 Penicillin G, guidelines for pediatric use, 215t Penicillin G (potassium), guidelines for pediatric use, 215t–216t Pericolace. See Docusate and casanthranol Peripheral nerve blocks, 229–234 Peripheral neuroectodermal tumors, secondary skeletal involvement in, 123 Perkin line, 62, 62f Peterson classification, of physeal injuries, 174, 176f Phenergan. See Promethazine Phenytoin, teratogenicity, 161 Phocomelia, in Cornelia de Lange syndrome, 140 Phosphorus, serum, normal values, 211t Physeal closure, age of, 9–17, 10f–11f Physical growth norms for boys, 34, 34f for girls, 34, 35f Pierre-Robin syndrome, and clubfoot, 94 Plagiocephaly, 106 Platyspondyly, 136 Poland syndrome clinical features, 158 and syndactyly, 158 Polio, 169t Polydactyly, 159–160 overgrowth syndromes and, 151 radial (preaxial), 159 ulnar (postaxial), 159 Polysyndactyly, 158 Polytrauma patient definition, 175 initial evaluation, 177 laboratory investigation in, 175 management, 175–178 management of, adjuncts in, 177–178 physical examination, 177–178 primary survey (ABCDE), 177 radiographic studies of, indications for, 175 secondary survey, 177 Ponsetti technique, for clubfoot cast, 241–244, 243f Popliteal block, 232–234 Popliteal (Baker) cyst, 89–91, 90f Popliteal pterygium syndrome, 148 Potassium, serum, normal values, 211t Power ratio, 18 Prader-Willi syndrome, 150–151 Prochlorperazine, guidelines for pediatric use, 221t Progressive diaphyseal dysplasia, 141 Promethazine, guidelines for pediatric use, 221t Proteus syndrome, 153 diagnostic criteria for, 153, 153t and macrodactyly, 103 Protrusio acetabuli, in Marfan syndrome, 144 Proventil. See Albuterol Proximal femoral focal deficiency, 75–77
classification (Aitken), 75–76, 75f Pseudoachondroplasia, 134–136 Psychomotor skills in infants and children ages 1 to 5, 2–3 referral criteria, 3 Pterygium syndromes, 148 Q 22q deletion syndrome, 161 R Radial clubhand, 159 Radial nerve, testing, 7 Radius distal, physeal fractures, 194–195 growth, 25f head and neck fractures, 185–186, 192 manipulation techniques for, 186 reduction, maintaining, 186 treatment, 186 Wilkins classification, 185 Monteggia fracture-dislocation, 186–187 Ranitidine HCL, guidelines for pediatric use, 221t Refsum disease, 170t Regional anesthesia, intravenous, 229 Regional nerve blocks, 229–234 Reimer migration index, 166, 168f Retropharyngeal space, 18, 18f Rhabdomyosarcoma, 123 Rib(s), tumors, 125 Rib-vertebral angle difference (RVAD) of Mehta, in scoliosis, 110, 111f Rifampin, guidelines for pediatric use, 216t Riley-Day familial dysautonomia, 140 Risser sign, 21, 21f, 110, 110f Rocephin. See Ceftriaxone Rod-body myopathy, 171t Roussy-Levy syndrome, 170t Russell-Silver syndrome, 152 RVAD. See Rib-vertebral angle difference (RVAD) of Mehta S Sacral agenesis, partial, with bladder exstrophy, 77 Sacroiliac joint aspiration or injection, technique for, 236–238, 237f infection, and back pain, 116 Saethre-Chotzen syndrome, 159 and syndactyly, 158 Salter-Harris classification, of physeal injuries, 174, 175f Sandifer syndrome, 105 SanFillipo syndrome, 158t Sauvegrain method (simplified), for skeletal maturity assessment, 14, 15f Scaphoid fractures, 194 Scapula, tumors, 126 Scheie syndrome, 158t Scheuermann kyphosis, 118–119, 122 and back pain, 116 Sclerosing bone disorders, 141–142 Scoliometer measurement, 108–109, 109f
256 Index Scoliosis adolescent, 106 treatment, 115 in cleidocranial dysplasia, 139 Cobb angle, 108–109 congenital, 117–118, 136, 137, 159, 163 definition, 117 types, 117 contractural syndromes and, 147–148 curve assessment, 107 Cobb measurement, 109–110, 110f thoracic, King classification, 112, 112f thoracolumbar, 112 curve types King classification, 112, 112f Lenke classification, 112, 113f differential diagnosis, 107 in Down syndrome, 162 forward-bend test for, 108, 108f history-taking for, 106 idiopathic, 106–115 treatment, 112–115 in Turner syndrome, 162 infantile, 106 treatment, 112–115 juvenile, 106 treatment, 115 in Klinefelter syndrome, 163 in Marfan syndrome, 144 in Noonan syndrome, 163 overgrowth syndromes and, 151, 152 physical findings and examination in, 106–107 radiographic assessment, 109–110 rib-vertebral angle difference of Mehta in, 110, 111f in Riley-Day familial dysautonomia, 140 rotation in, estimation in adolescent (Nash and Moe method), 110, 111f in infantile or juvenile patient, 110, 111f scoliometer measurement, 108–109, 109f screening for, 108–109 Seatbelt injury, spinal, 202, 203f Second rocker, definition, 51 Sensation, 3 Septic arthritis, microbiology, 129, 129t Serum glutamic-oxaloacetic transaminase (SGOT). See Aspartate aminotransferase Serum glutamic-pyruvic transaminase (SGPT). See Alanine aminotransferase Sever disease, 122 Short stature skeletal dysplasia and, 134 syndromes involving, 140–141 Shoulder injury(ies), 178–181 birth fractures as, 179 Shprintzen-Goldberg syndrome, 146 Sickle cell anemia, 150 Sickle cell trait, 150 Sickle crisis, 150 Skeletal development, 9–17 Skeletal dysplasia, 134–139 and congenital scoliosis, 118 Skeletal maturity, assessment
during accelerating pubertal growth phase, 21, 21f Modified Tanner-Whitehouse III Assessment, 14, 16t, 17f olecranon method, 21, 21f Sauvegrain method (simplified), 14, 15f Skeletal traction, pin placement for, 225, 226f Skewfoot, 147 Skin traction application, 224, 225f indications for, 224 Slipped capital femoral epiphysis, 71–74 acute, 72 chronic, 72 classification, 72 clinical presentation, 72 complications, 73 in Down syndrome, 162 epidemiology, 71 etiology, 71–72 grading, 72 radiographic features, 72–73 treatment, 73 Sly syndrome, 158t Sodium, serum, normal values, 211t Spina bifida, 173 associated abnormalities, 173 motor classification, 173 treatment, 173 Spinal deformity, syndromes with, 160–161 Spinal dysraphism and clubfoot, 94 and congenital scoliosis, 117 Spinal growth, 20–21 guidelines, 20–21 rules of thumb, 20–21 T1-S1, 20–21 velocity, by segments, 21, 22f Spinal growth remaining, calculation, 21, 23f–24f Spinal muscular atrophy type I (Werdnig-Hoffmann), 169t type II, 169t type III (Kugelberg-Welander), 169t Spine flexion-distraction (Chance) (seatbelt) injuries, 202, 203f fractures, 195–202 tumors, 125 Spinocerebellar degeneration. See Friedreich ataxia Spondyloepiphyseal dysplasia, and clubfoot, 94 Spondyloepiphyseal dysplasia congenita, 136 Spondyloepiphyseal dysplasia tarda, 137 Spondylolisthesis, 119–122 classification, 119 epidemiology, 119 etiology, 119 imaging, 121 plain radiographic findings in, 120f, 121, 121f risk factors for, 119–120 signs and symptoms, 120–121 slip angle (lumbosacral kyphosis) in, measurement, 121, 121f treatment, 121–122 vertebral body slippage in, measurement, 120f, 121
Index 257 Spondylolysis, 119–122 and back pain, 115 classification, 119 imaging, 121 isthmic, risk factors for, 119–120 plain radiographic findings in, 120f, 121, 121f risk factors for, 119–120 signs and symptoms, 120–121 slip angle (lumbosacral kyphosis) in, measurement, 121, 121f treatment, 121–122 vertebral body slippage in, measurement, 120f, 121 Sprengel anomaly, and congenital scoliosis, 118 Sprengel deformity, 173 Klippel-Feil syndrome and, 152 Stance phase, definition, 51 Stature. See Short stature; Stature-for-age norms Stature-for-age norms for boys, 34, 34f for girls, 34, 35f Steinberg sign, in Marfan syndrome, 143–144, 144f Steinert disease, 172t Sternoclavicular injury(ies), 181 Sternocleidomastoid muscle, contracture, 105, 106 Stickler syndrome, 146 Streeter dysplasia. See Congenital constriction band syndrome Streeter’s bands. See Congenital constriction band syndrome Stulberg rating, of outcomes with Legg-CalvePerthes disease, 67, 68f Sturge-Weber syndrome, 149 Symphalangism, 136 Syndactyly, 157–159 complete, 157 complex, 157 congenital constriction bands and, 132 isolated, 158 partial, 157 simple, 157 T Talipes equinovarus. See Clubfoot Talocalcaneal angle anteroposterior, 50f, 51 lateral, 50–51, 50f Talocalcaneal bar, 96, 97f Talonavicular joint, dorsolateral dislocation, 99–100, 100f Talus, congenital vertical, 99–100, 100f Tanner-Whitehouse III Skeletal Maturity Assessment (simplified), 14, 16t, 17f Tarsal coalition, 96, 97f Tarsal navicular, ossification, 12 TAR syndrome, 159 Tegopen. See Cloxacillin Teratogen(s), syndromes caused by, 161 Tethered cord, 173 and back pain, 116 and clubfoot, 94 Tetracycline HCL, guidelines for pediatric use, 216t
Thigh-foot angle, 48 measurement, 46f, 47f, 48 Third rocker, definition, 51 Thomsen disease, 172t Thoracic spine, fractures, 202 Thumb gamekeeper’s, 194 metacarpal fractures, 194 Thumb sign, in Marfan syndrome, 143–144, 144f Tibia congenital dysplasia (anterolateral bow/ pseudarthrosis), 91–92, 92f distal growth, 25, 25f growth remaining curves, by skeletal age for boys, 29f, 30 for girls, 29f, 30 normal growth, 207 physeal arrest, 208–209 physeal fractures, growth plate damage in, 30 hemimelia, 55 and clubfoot, 94 length, normal for boys, 27f for girls, 28f posteromedial bow, 92–93, 93f proximal epiphysis, ossification, 9 growth, 25, 25f growth remaining, by skeletal age, 26f physeal injury, 206 physis, normal anatomy and growth, 204 traction pin placement in, 225, 226f tubercle fractures, 206–207 Tibial joint angle, 43, 44f Tibia vara, 79–83 adolescent, 79, 79t treatment, 82 differential diagnosis, 81 infantile, 79–80, 79f, 79t medial plateau depression in, 80, 81f treatment, 81–82 juvenile, 79, 79t Langenskjold stages, 79–80, 79f mechanical axis deviation in, 81, 82f metaphyseal-diaphyseal angle in, 80, 80f radiographic features, 79–81, 79f–81f recurrence risk, metaphyseal slope and, 80, 80f treatment, 81–82 Tibiofemoral angle, development, during growth, 43, 43f Ticarcillin, guidelines for pediatric use, 216t Tigan. See Trimethobenzamide Tillaux fractures, 208 Tobramycin, guidelines for pediatric use, 216t Toe walking, 102–103 idiopathic, 102 Toradol. See Ketorolac Torticollis, 105–106 and developmental dysplasia of the hip, 59 muscular, 105–106 Traction. See also Skeletal traction; Skin traction modified Bryant’s, 226, 227f ninety-nine, 226, 228f split Russell’s, 226, 227f
258 Index Transmalleolar axis, 47f, 48 Trauma, pediatric, 174–209. See also Child abuse; Fracture(s); Polytrauma patient and back pain, 116 blunt, in asymptomatic patient, evaluation, 196f obtunded patient with, evaluation, 199f symptomatic patient with, evaluation, 198f temporarily unassessable patient with, evaluation, 197f Trevor disease, 138 Trimethobenzamide, guidelines for pediatric use, 221t Triplane fracture, 208 Triradiate cartilage, closure, 12, 21 Trisomy 21. See Down syndrome Tuberculosis, and back pain, 116 Tumor(s) and back pain, 116 in long bones, 124f, 125 musculoskeletal, 123–126 abbreviations for, 127t benign, 123–125, 124f staging, 126 computed tomography of, 123 magnetic resonance imaging of, 123 malignant, 123, 124f, 125 staging, 126, 126t plain films of, 123, 124f radionuclide scans of, 123 sites of, in children, 123, 124f types, 123–125, 127t pelvic, 125–126 of ribs, 125 scapular, 126 spinal, 125 vertebral body, 125 Tums. See Calcium carbonate Turner syndrome, 162 Tylenol. See Acetaminophen U UBC. See Unicameral bone cyst Ulna growth, 25f and humerus, normal alignment, 182f Ulnar bow sign, 186, 187f Ulnar clubhand, 160 Ulnar nerve, testing, 7, 8f Ultracef. See Cefadroxil Ultrasound, for diagnosis of developmental dysplasia of the hip, 59–62, 61f Unicameral bone cyst, 124, 124f proximal humeral, fracture through, 180–181
Upper extremity abnormalities, in Cornelia de Lange syndrome, 140 contributions to growth of long bones, 22, 25f growth remaining calculation, 33, 33f motor examination, 3–4, 5f muscle innervation, 4, 6f muscle strength, grading, 4 osteochondrosis, 122 peripheral nerve testing in, 7, 8f sensory and motor innervation, 5f V VACTERL, 160–161 and congenital scoliosis, 117 VACTERLS, 160–161 Valgus deformity, lower-limb, mechanical axis deviation in, 81, 82f Valium. See Diazepam Vancomycin, guidelines for pediatric use, 216t Vascular abnormalities, 148–150 VATER, 152, 159, 160–161 and congenital scoliosis, 117 Ventolin. See Albuterol Versed. See Midazolam Vertebral body, tumors, 125 Vertebral endplate, herniated, and back pain, 116 Vital signs, normal, by age, 177, 177t Vitamin C. See Ascorbic acid W Walker-Murdoch sign, in Marfan syndrome, 143–144, 144f Warfarin, teratogenicity, 161 WBC. See White blood cell count (WBC) Weight-for-age percentiles for boys, 34, 34f for girls, 34, 35f Werdnig-Hoffmann disease, 169t Whistling face syndrome. See Freeman-Sheldon syndrome White blood cell count (WBC), normal, by age, 210t Wildervanck syndrome, 161 Wilms tumor, hemihypertrophy and, 152 Wrist sign, in Marfan syndrome, 143–144, 144f Z Zantac. See Ranitidine HCL Zinacef. See Cefuroxime Zofran. See Ondansetron